Académique Documents
Professionnel Documents
Culture Documents
TONY BURNS
MB BS (Lond), FRCP
Emeritus Consultant Dermatologist
The Leicester Royal Infirmary, Leicester
Eighth Edition
Blackwell
Publishing
To all medical students, and t o our children:
James, Matthew, John Joseph and David.
C3 1965, 1969, 1973, 1977, 1983, 1990, 1996, 2002 by Blackwell Science Ltd
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First published 1965 Fourth edition 1977 Seventh edition 1996 Reprinted 2003, 2004, 2005
Second edition 1969 Fifth edition 1983 Reprinted 2001
Third edition 1973 Sixth edition 1990 Eighth edition 2002
lSBN 0-632-06494-3
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Preface, iv
Acknowledgements, v
Index, 185
iii
In this, the eighth edition of Lecture Notes on Dermatology, we have updated the text
with particular regard t o advances in treatment. Numerous tables of salient points
provide ready reference but, as in previous editions, we have attempted t o create a
'user-friendly' readability.
W e hope that the book will be of value not only t o medical students, but also t o
general practitioners, and nurses involved in the care of dermatology patients. W e
also hope that exposure t o Lecture Notes on Dermatology will prompt a deeper
interest in this important medical specialty.
Robin Graham-Brown
Tony Burns
Acknowledgements
We would like to thank Drs lmrich Sarkany and Charles Calnan, under whose guid-
ance we both learned dermatology, and we are grateful to them for some of the
illustrations.We are also grateful t o our students, who remind us constantly of the
importance of clarity in communication. Finally, we thank the staff at Blackwell
Publishingwho have helped us through the editingand production stages.
This page intentionally left blank
Structure and Function of
the Skin, Hair and Nails
yourself you are rubbing off small balls of kera- rate, and persist through the full thickness of the
tin -which has built up because of your insani- epidermis. The main stimulus t o melanin pro-
tary habits.When a plaster cast is removed from duction is ultraviolet (UV) radiation. Melanin
a fractured limb after several weeks in situ there protects the cell nuclei in the epidermis from
is usually a thick layer of surface keratin, the the harmful effects of UV radiation. A sun tan is
removal of which provides hours of absorbing a natural protective mechanism, not a cosmetic
occupational therapy. boon! Skin neoplasia is extremely uncommon in
Look at the layers more closely (Fig. I.I).The dark-skinned races because their skin is pro-
basal layer is composed of columnar cells which tected from UV damage by the large amounts of
are anchored t o a basement membrane-a mul- melanin i t contains.This is not the case in the
tilayered structure from which anchoring fibrils pale, pimply lager-swilling advert for British
extend into the superficial dermis. Interspersed manhood who dashes onto the beach in Ibiza
among the basal cells are melanocytes-large and flash-fries himself t o lobster thermidor on
dendritic cells derived from the neural crest- day one of his annual holiday.
which are responsible for melanin pigment The prickle cell layer acquires its name from
production. Melanocytes contain cytoplasmic the spiky appearance produced by intercellular
organelles called melanosomes, in which bridges (desmosomes) that connect adjacent
melanin is synthesized from tyrosine. The cells. Scattered throughout the prickle cell layer
melanosomes migrate along the dendrites of are Langerhans' cells. These dendritic cells are
the melanocytes, and are transferred t o the probably modified macrophages, which origin-
keratinocytes in the prickle cell layer. In white ate in the bone marrow and migrate t o the epi-
people the melanosomes are grouped together dermis.They are the first line of immunological
in membrane-bound 'melanosome complexes', defence against environmental antigens, and are
and they gradually degenerate as the ker- responsible for the uptake of such antigens and
atinocytes move towards the surface of the their presentation t o immunocompetent lym-
skin. In black people, the skin contains the same phocytes so that an immune response can be
number of melanocytes as that of white people, mounted.
but the melanosomes are larger, remain sepa- Above the prickle cell layer is the granular
Skin structure 3
taining numerous darkly staining particles Apocrine sweat glands are found principally in
known as keratohyalin granules. Also present in the axillae and anogenital region. Specialized
the cytoplasm of cells in the granular layer are apocrine glands include the wax glands of the
organelles known as lamellar granules (Odland ear and the milk glands of the breast. Apocrine
bodies). These contain lipids and enzymes, and glands are also composed of a secretory coil
they discharge their contents into the intercel- and a duct, but the duct opens into a hair follicle,
lular spaces between the cells of the granular not directly onto the surface of the skin. Apo-
layer and stratum corneum-providing the crine glands produce an oily secretion contain-
equivalent of 'mortar' between the cellular ing protein, carbohydrate, ammonia and lipid.
'bricks', and contributingt o the barrier function These glands become active at puberty, and
of the epidermis. secretion is controlled by adrenergic nerve
The cells of the stratum corneum are flat- fibres. Pungent axillary body odour (axillary
tened, keratinized cells which are devoid of bromhidrosis) is the result of the action of bac-
nuclei and cytoplasmic organelles. Adjacent teria on apocrine secretions. In some animals
cells overlap at their margins, and this locking apocrine secretions are important sexual at-
together of cells, together with intercellular tractants, but the average human armpit pro-
lipid, forms a very effective barrier.The stratum vides a different type of overwhelming olfactory
corneum varies in thickness according t o the experience.
region of the body. It is thickest on the palms of
the hands and soles of the feet. Hair
Hairs grow out of tubular invaginations of the
Epidermal appendages epidermis known as follicles, and a hair follicle
The eccrine and apocrine sweat glands, the hair and its associated sebaceous glands are referred
and sebaceous glands, and the nails, constitute t o as a 'pilosebaceous unit'. There are three
the epidermal appendages. types of hair: fine, soft lanugo hair is present in
utero and is shed by the eighth month of fetal life;
Eccrine sweat glands vellus hair is the fine downy hair which covers
Eccrine sweat glands are important in body most of the body except those areas occupied
temperature regulation. A human has between by terminal hair; thick and pigmented term-
two and three million eccrine sweat glands cov- inal hair occurs on the scalp, eyebrows and
ering almost all the body surface.They are par- eyelashes before puberty-after puberty, under
ticularly numerous on the palms of the hands the influence of androgens, secondary sexual
and soles of the feet. Each consists of a secret- terminal hair develops from vellus hair in the
ory coil deep in the dermis, and a duct that con- axillae and pubic region, and on the trunk and
veys the sweat t o the surface. Eccrine glands limbs in men. O n the scalp, the reverse occurs
secrete water, electrolytes, lactate, urea and in male-pattern balding-terminal hair becomes
ammonia.The secretory coil produces isotonic vellus hair under the influence of androgens. In
sweat, but sodium chloride is reabsorbed in the men, terminal hair on the body usually increases
duct so that sweat reachingthe surface is hypo- in amount as middle age arrives, and hairy ears
tonic. Patients sufferingfrom cystic fibrosis have are a puzzling accompaniment of advancing
defective resorption of sodium chloride, and years. One struggles t o think of any biological
rapidly become salt depleted in a hot environ- advantage that hairyears might confer.
ment. Eccrine sweat glands are innervated by Hair follicles extend into the dermis at an
the sympathetic nervous system, but the neuro- angle (Fig. 1.2). Asmall bundle of smooth muscle
transmitter is acetylcholine. fibres, the arrector pili muscle, is attached t o
the side of the follicle.
Arrector pili muscles are supplied by adren-
4 Chapter I: Structure and Function of the Skin, Hair and Nails
genetically determined, and ranges from 2 t o glands are part of the pilosebaceous unit, and
more than 5 years.This is why some women can their lipid-rich product (sebum) flows through a
grow hair down t o their ankles, whereas most duct into the hair follicle. They are holocrine
have a much shorter maximum length. Scalp hair glands -sebum is produced by disintegration of
catagen lasts about 2 weeks and telogen from 3 glandular cells rather than an active secretory
t o 4 months.The daily growth rate of scalp hair process. Modified sebaceous glands which open
is approximately 0.45 mm. The activity of each directly on the surface are found on the eyelids,
follicle is independent of that of its neighbours, lips, nipples, glans penis and prepuce, and the
which is fortunate, because if follicular activity buccal mucosa (Fordyce spots).
was synchronized, as it is in some animals, we Sebaceous glands are prominent at birth,
would be subject t o periodic moults, thus under the influence of maternal hormones, but
adding another dimension t o life's rich tapestry. atrophy soon after, and do not enlarge again
A t any one time approximately 85% of scalp until puberty. Enlargement of the glands and
hairs are in anagen, I % in catagen and 14% in sebum production at puberty are stimulated by
telogen. The average number of hairs shed androgens. Growth hormone and thyroid hor-
daily is 100. In areas other than the scalp anagen mones also affect sebum production.
is relatively short-this is also fortunate, be-
cause if it was not so, we would all be kept Nails
busy clipping eyebrows, eyelashes and nether A nail is a transparent plate of keratin derived
regions. from an invagination of epidermis on the dor-
It is a myth that shaving increases the rate of sum of the terminal phalanx of a digit (Fig. 1.4).
growth of hair and that it encourages the devel- The nail plate is the product of cell division in the
opment of'thicker' hair; nor does hair continue nail matrix, which lies deep t o the proximal nail
growing after death-shrinkage of soft tissues fold, but is partly visible as the pale 'half-moon'
around the hair produces this illusion. (lunula) at the base of the nail. The nail plate is
Human hair colour is produced by two types firmly adherent t o the underlying nail bed.The
of melanin-eumelanins in black and brown cuticle is an extension of the horny layer of the
hair, and phaeomelanins in auburn and blond
hair.
Greying of hair is the result of a decrease in
tyrosinase activity in the melanocytes of the hair
bulb. The age of onset of greying is genetically
determined, but other factors may be involved
such as auto-immunity-premature greying of
the hair is a recognized association of perni-
cious anaemia.The phenomenon of'going white
overnight', usually attributed t o a severe fright,
is physically impossible. It is, however, possible
t o 'go white' over a period of a few days as a re-
sult of selective loss of remaining pigmented
hairs in someone who has extensive grey hair-
this occurs in one type of alopecia areata.
Sebaceous glands
Sebaceous glands are found everywhere on the
skin apart from the hands and feet.They are par-
ticularly numerous and prominent on the head
and neck, the chest, and the back. Sebaceous Fig.1.4 The nail.
6 Chapter I: Structure and Function ofthe Skin, Hair and Nails
proximal nail fold onto the nail plate. I t forms a mediators such as histamine, prostaglandins.
seal between the nail plate and proximal nail leul<otrienes and eosinophil and neutrophii
fold, preventing penetration of extraneous chemotactic factors. Macrophages are phago-
material. cytic cells that originate in the bone marrow,
Nail growth is continuous throughout life, and they act as scavengers of cell debris and
but is more rapid in youth than in old age.The extracellular material.
average rate of growth of fingernails is approxi- The dermis is also richly supplied with
mately I mm per week, and the time taken for a blood vessels, iymphatics, nerves and sensory
fingernail t o grow from matrix t o free edge is receptors.
about 6 months. Nails on the dominant hand Beneath the dermis, a layer of subcutaneous
grow slightly more rapidly than those on the fat separates the skin from underlying fasciaand
non-dominant hand.Toenails grow at one-third muscle.
the rateoffingernails,and taleabout 18 months
t o grow from matrixto free edge. Dermatoglyphics
Many factors affect nail growth rate. It is ln- Fingerprints, the characteristic elevated ridge
creased in psoriasis, and may be speeded up in patterns on the fingertips of humans, are unique
the presence of inflammatory change around t o each individual.The fingers and toes, and the
the nail. A severe systemic upset can produce a palms and soles, are covered with a system of
sudden slowing of nail growth, causing a trans- ridges which form patterns.The term'dermato-
verse groove in each nail plate.These grooves, glyphics' is applied t o the configuration of the
Iknown as Beau's lines, subsequently become ridges. If you look closely at your hands you will
visible as the nails grow out. Nail growth may see these tiny ridges, which are separate from
also be considerably slowed in the digits of a the skin creases. O n the tips ofthe fingers there
limb immobilized in plaster. are three basic patterns: arches, loops and
whorls (Fig. I.S).The loops are subdivided into
The dermis ulnar o r radial, depending on whether the loop
The dermis is a layer of connective tissue lying is open t o the ulnar o r radial side ofthe hand.A
beneath the epidermis, and forms the bull< of triangular intersection of these ridges is known
the skin.The dermis and epidermis interdigitate as a triradius,and these triradii are notonly pre-
via downward epidermal projections (rete sent on fingertips, but also at the base of each
ridges), and upward dermal projections (dermal finger, and usually on the proximal part of the
papillae) (Fig. I .2).The main feature of the der- palm.
mis is a nenvorkofinrerlacingfibres, mostly col- N o t only are the ridge patterns of finger-
lagen, but with some elastin.These fibres give prints useful for the identification and convic-
the dermis great strength and elasticity. The tion of those who covet their neighbours'
collagen and elastin fibres, which are protein, goods, but characteristic dermatoglyphic ab-
are embedded in a ground substance of mu- normalities frequently accompany many chro-
copolysaccharides(glycosaminoglycans). mosomal aberrations.
The main cellular elements of the dermis are
fibrobiasts, mast cells and macrophages. Fibro-
blasts synthesize the connectivetissue matrix of
the dermis, and are usually found in close prox-
imitytocollagenandelarrin fibres. Mastcellsare Skin is like wax paper that holds everythingin
specialized secretory cells present throughout without dripping. ( A r t Linkletter, A Child's
the dermis, but they are more numerous Garden ofMisinformotion, 1965)
.
around blood vessels and appendaner.
, - They It is obvious from the complex structure of the
contain granules whose contents include skin that it is not there simply t o hold all the
Functions of the skin 7
other bits of the body together. Some of the organ and plays an important role in host
functions of skin are as follows: defence. N o t only Langerhans' cells but also
keratinocytes prepare external antigens for
presentation t o T lymphocytes, which then
mount an immune response.
The protective effect of melanin against UV
* Prevents loss ofessentialbody fluids damage has already been mentioned.
* Protects againstentryoftoxic The skin is a vital part of the body's tem-
environmental chemicals and perature regulation system. The body core
microorganisms temperature is regulated by a temperature-
* Immunolagicalfunctions sensitive area in the hypothalamus, and this is
-
* Protects against damage from Wradiation
Regulates bodytemperature
* Synthesis afvitaminD
influenced by the temperature of the blood
which perfuses it. The response of the skin t o
* Important in sexual attraction and social cold is vasoconstriction and a marked reduction
~
The skin is also a huge sensory receptor, per- In addition t o all these mechanistic functions,
ceivingheat, cold, pain, light touch and pressure, the skin plays an essential aesthetic role in social
and even tickle. As you are probably still grap- interaction and sexual attraction.
pling with the conundrum of the biological sig- Hence, you can see that your skin is doing
nificance of hairy ears in the elderly male, t r y a good job. Apart from looking pleasant, i t is
switching your thoughts t o the benefits of tickly saving you from becoming a cold, UV-damaged,
armpits! brittle-boned, desiccated'prune'.
Approach to the Diagnosis
ofDevmatological Disease
-
~
-
Occupation andhobbies
The skinis frequently affectedby materials
encountered atwork and in the home
Watch out, too, for the very high expecta-
tions of many patients. They know that visible
evidence is there for all t o see: dermatology
Examination II
-eczema
Thecolaurltisalwaysuseful tonotethe
colour: red,purple.brawn. slate-black.etc.
* Scrapingapsoriatic plaque for capillay
bleeding
* The Nikolskysigninblisteringdiseases
* Surface features (Table 2.1).It is helpful to * 'Diascopy'in suspected cutaneous
assesswhetherthesurfaceissmoothor tuberculosis
I2 Chapter 2: Diagnosis of Dermatological Disease
-
* M a d e : a flat, circumscribed area of skin discoloration
Papule: acircumscribedelwatianoftheskinlessthan0.5mindiameter
* Nodule: acircumscribedvisiblearpalpablelump,largerthan0.5cm
Plaque: acircumscribed, disc-shaped, elevated area ofskin:
'small'Qcm in diameter
--
* Bulk a largevisiblecollection offluid(over0.scm)
Pustule: avisibleaccumulationofpus
Ulcer: a loss of epidermis (often with loss ofunderlyingdermis and subcutis as well)
* Weal: a circumscribed. elevated areaofcutaneous oedema
--
Surface characteestic'es
Scale: visible and palpable flakes due to aggregation andlor abnormalities ofshed epidermal cells
Crust: accumulateddriedexudate,e.g.semm
-
* Horn: an elevatedprojectionofkeratin
Excoriation: asecondary,superficiaI ulceration.due to scratching
* Maceration: an appearance ofsurface softeningdue to constantwetting
Lichenification:aflat-toppedthickeningofthe skin often secondaryto scratching.
~ ~ ~~ ~ ~~ ~~~ ~~ ~ ~
I t is fair t o say that in inflammatory der- These situations are covered later in the appro-
matosesacomplication is havingtodecide which priate chapters.The advances in modern genet-
lesion or lesions to select for this descriptive ics, too, mean that blood (or other tissues) can
process. Skin diseases are dynamic. Some le- be analysed for evidence of specific defects.
sions in any rash will be very early, some very Sometimes clinical tindings alone will not pro-
late, and some at various intermediate evolu- duce a satisfactory working diagnosis, o r fur-
tionary stages. ther information is required in order t o plan
Try t o examine as many patients as you can: optimal management.
frequent exposure t o skin diseases develops an A number of important techniques are avail-
ability t o recognize those lesions which provide able t o provide further information. Some of
the most useful diagnostic information, these, such as appropriate blood tests and
This diagnostic process willgradually become swabs for bacteriology and virology, should be
one that you will perform increasingly easily and familiar from other branches of medicine, and
confidently as experience develops. are fully covered in other introductory text-
books. Others, however, are more specific t o
dermatological investigation. Common, useful
investigations in sldn diseases include the
following:
Inevitably, history and examination alone will Blood tests-for underlying systemic
not always provide all the information required. abnormalities and, increasingly, for genetic
Thereare some sldn disorders in which further analysis.
investigation is nearly always necessary: either Swabs and other samples-for infections.
t o confirm a diagnosis with important prognos- Wood's light-some disorderslfeatures are
tic o r therapeutic implications (e.g. blistering easier t o see.
disorders), o r t o seek an underlying, associated Skin scrapes o r nail clippings-microscopy
systemic disorder (e.g. in generalized pruritus). and mycological culture.
Investigation 13
which can result in inadequate deeper excision samples and is only appropriate for diagnostic
(Fig. 2.3). biopsies o r removing tiny lesions (see Fig.
3 Repair the defect. Edges left by either inci- 2.4a-c):
sional o r excisional biopsy are brought neatly I Administer local anaesthesia (see above).
together with sutures; the choice of suture 2 Push the punch biopsy blade into the lesion
material is not critical, but for the best cosmetic using a circular motion.
result use the finest possible, preferably a 3 Lift out the small plug, and separate with
synthetic monofilament suture (e.g. prolene). scissors o r a scalpel blade.
Note: if there will be significant tension on the 4 Achieve haemostasis with silver nitrate o r a
suture line, consider asking a trained plastic o r small suture.
dermatological surgeon for advice.
Patch tests
Punch biopsy If a contact allergic dermatitis is suspected, a
This is much quicker, but produces small patch test is performed. In this process possible
Investigation 15
Bacterialinfectians, 17 Viralinfections, 22
A mighty creature is the germ fer from leg oedema of cardiac, venous o r lym-
Though smaller than the pachyderm phaticorigin.
His customary dwelling place The affected area becomes erythematous,
Is deep within the human roce hot and swollen (Fig. 3.l), and blister formation
His childish pride he often pleases and areasofskin necroris mayoccur.The patient
By giving peoplestrange diseases is pyrexial and feels unwell. Rigors may occur
Do you,my poppet, feel infirm? and, in the elderly, a toxic confusional state.
Yau probably contain agerm In presumed streptococcal cellulitis penicillin
(Ogden Nash, The Germ) is the treatment of choice, initially given as ben-
r/lpenicillin intravenously. If the leg is affected,
bed rest is an important aspect of treatment.
Bacterial infections Where there is extensive tissue necroris, surgi-
cal debridement may be necessary.
Streptococcal infection A particularly severe, deep form of cellulitis,
Cellulitis involvingfasciaand muscles, is known as'necro-
Cellulids isa bacterial infection of subcutaneous tizingfasciitis'.Thir disorder achieved notoriety
tissues which, in immunologically normal a few years ago when it attracted the attention
individuals, is usually caused by Streptococcus of the UK popular press and was described as
pyogenes.'Erysipelas' is a term applied t o super- being caused by a'flerh-eatingvirus'. I t is assocl-
ficial streptococcal cellulitis which has a well- ated with extensive tissue necroris and severe
demarcated edge. Occasionally, other bacteria toxaemia, and is rapidly fatal unless urgenttreat-
areimplicated in cellulitis-Hoemophilusinpuen- ment, including excision of the affected area, is
zoe is an important cause of facial cellulitis in undertaken.
children, often in association with ipsilateral Some patients have recurrent episodes of
otitis media. In immunocompromised individu- cellulitis, each episode damaging lymphaticsand
als, a variety of bacteria may be responsible for leading t o further oedema.These cases should
cellulitis. be treated with prophylactic oral penicillinv o r
Cellulitis frequently occurs on the legs, but erythromycin, t o prevent further episodes.
other parts of the body may be affected-the
face is a common site for erysipelas.Thc organ- Staphylococcal infection
isms may gain entry into the skin via minorabra- Folliculitis
sions, o r fissures between the toes associated Infection of the superficial part of a hair follicle
with tinea pedis, and leg ulcers provide a portal with Staphyiococcus aureus produces a small pus-
of entry in many cases. A frequent predisposing tule on an erythematous base, centred on the
factor is oedema of the legs, and cellulitis is a follicle.
common condition in the elderly,who often suf- Folliculitis is a common problem in eczema
18 Chapter 3: Bacterial and Viral Infections
Carbuncle
A carbuncle is a deep infection of a group of ad-
jacent hair follicles with S.aureus. A frequent site
for a carbuncle is the nape of the neck. Initially,
the lesion is a dome-shaped area of tender ery-
thema, but after a few days suppuration begins,
and pus is discharged from multiple follicular
orifices. Carbuncles are usually encountered in
middle aged and elderly men, and are associated
with diabetes and debility.They are uncommon
nowadays. Flucloxacillin should be given for
treatment.
Impetigo
This is a contagious superficial infection which
occurs in two clinical forms, non-bullous and
bullous. Non-bullous impetigo is caused by S.au-
Fig. 3.1 Cellulitis. reus, streptococci, o r both organisms together.
The streptococcal form predominates in warm,
humid climates, for example the southern USA.
patients treated with ointment-base topical Bullous impetigo is caused by S. aureus. Lesions
steroid preparations. may occur anywhere on the body. In the non-
Mild folliculitis can be treated with a topical bullous form the initial lesion is a small pustule
antibacterial agent, but if it is extensive a sys- which ruptures t o leave an extending area of
temic antibiotic may be required. exudation and crusting (Fig. 3.2). The crusts
eventually separate t o leave areas of erythema,
Furunculosis ('boils') which fade without scarring. In the bullous
A boil (furuncle) is the result of deep infection of form, large, superficial blisters develop. When
a hair follicle by S. aureus. A painful abscess de- these rupture there is exudation and crusting,
velops at the site of infection, and over a period and the stratum corneum peels back at the
of a few days becomes fluctuant and'points' as a edges of the lesions.
central pustule. Once the necrotic central core Streptococcal impetigo may be associated
has been discharged, the lesion gradually re- with poststreptococcal acute glomeruloneph-
solves. In some patients boils are a recurrent ritis.
problem, but this is rarely associated with a sig- Impetigo may occur as a secondary phenom-
nificant underlying disorder. Such individuals enon in atopic eczema, scabies and head louse
may be nasal o r perinea1 carriers of staphylo- infection.
cocci, and organisms are transferred on the In localized infection, treatment with a topi-
digits t o various parts of the body. cal antibiotic such as mupirocin will suffice, but
Patients sufferingfrom recurrent boils should in more extensive infection treatment with a
have swabs taken from the nose for culture, and systemic antibiotic such as flucloxacillin o r ery-
if found t o be carrying staphylococci should be thromycin is indicated.
Bacterial infections 19
the leprosy bacillus, Mycobaaerium leprae, in ripheral nerves are affected. Skin lesions are sin-
1873, and if the possibility of leprosy enters into gle, o r few in number, and are well defined.They
the discussion of differential diagnosis in the are macules o r plaques which are hypopig-
clinic, the eponymous title of this condition mented in dark skin.The lesionsare anaesthetic,
should always be used, because the fear of lep- sweating is absent, and hairs are reduced in
rosy is so ingrained, even in countries where it is number.Thickened branches of cutaneous sen-
not endemic. sory nerves may be palpable in the region of
Leprosy has a wide distribution throughout these lesions, and large peripheral nerves may
the world, with the majority of cases occurring also be palpable. The lepromin test is strongly
in the tropics and subtropics, but population positive. Histology shows well-defined tubercu-
movements mean that the disease may be en- loid granulomas, and bacilli are not seen on
countered anywhere. modified Ziehl-Nielsen staining.
Leprosy is a disease of peripheral nerves, but When the cell-mediated immune response is
i t also affects the skin, and sometimes other tis- poor, the bacilli multiply unchecked and the pa-
sues such as the eyes, the mucosa of the upper tient develops lepromatous leprosy. The bacilli
respiratory tract, the bones and the testes. Al- spread t o involve not only the skin, but also the
though it is infectious, the degree of infectivity is mucosaof the respiratory tract, the eyes, testes
low. The incubation period is lengthy, probably and bones. Skin lesions are multiple and nodular.
several years, and it is likely that most patients The lepromin test is negative. Histology shows a
acquire the infection in childhood. A low inci- diffuse granuloma throughout the dermis, and
dence of conjugal leprosy (leprosy acquired bacilli are present in large numbers.
from an infected spouse) suggests that adults In between these two extreme,'polar' forms
are relatively non-susceptible.The disease is ac- of leprosy is a spectrum of disease referred t o as
quired as a result of close physical contact with borderline leprosy, the clinical and histological
an infected person, the risk being much greater features of which reflect different degrees of
for contacts of lepromatous cases-the nasal cell-mediated responset o the bacilli.There is no
discharges of these individuals are the main absolute diagnostic test for leprosy-the diag-
source of infection in the community. nosis is based on clinical and histological
The clinical pattern of disease is determined features.
by the host's cell-mediated immune responset o Tuberculoid leprosy is usually treated with a
the organism. When this is well developed tu- combination of dapsone and rifampicin for 6
berculoid leprosy occurs, in which skin and pe- months; lepromatous leprosy with dapsone,
22 Chapter 3: Bacterial and Viral Infections
rifampicin and clofazimine for at least 24 W a r n are benign epidermal neoplasms caused
months.The treatment of leprosy may be com- by viruses of the human papillomavirus (HPV)
plicated by immunologically mediated'reaction- group.There are a number o f different strains o f
al states', and should besupervised bysomeone HPV which produce different clinical types of
experienced in leprosy management. warts. Warts are also lknown as 'verrucae', al-
though the term verruca in popular usage is
usually reserved for the plantarwart.
Common warts
These are raised, cauliflower-like lesions which
occur most frequently on the hands (Fig. 3.6).
They are extremely common in childhood and
early adult life.They may be scattered, grouped
o r periungual in distribution. Common warts in
children usually resolve spontaneously.
Common wartsareusually treated with wart
paints o r cryotherapy. Preparations containing
salicylic acid o r glutaraldehyde are often quite
effective, and a wart paint should certainly be
usedfor atleast 3 months beforeconsideringal-
ternative treatment.
Cryotherapywith liquid nitrogen can be used
on warts which do not respond t o wart paints.
A simple applicator of cotton wool wrapped
around the end o f an orange stick may be used.
This is dipped in the liquid nitrogenand then ap-
plied t o thewart until itand a narrow rim of sur-
Viral infections rounding skin are frozen. Alternatively, a liquid
nitrogen cryospray may be used.This is a painful
Warts procedure, and should n o t be inflicted on small
Fastingspittle isgoodfor warts. (Traditional children -however, most tiny tots will, sensibly,
English remedy) retreat under the desk protesting loudly at first
Viral infections 23
Plantar warts
Plantar warts may be solitary, scattered over
the sole of the foot, o r grouped together pro-
ducing so-called 'mosaic' warts (Fig. 3.7). The
typical appearance is of a small area of thickened
skin which, when pared away, reveals numerous
small black dots produced by thrombosed
capillaries. Plantar warts are frequently painful.
They must be distinguished from calluses and
corns, which develop in areas of friction over
bony prominences. Calluses are patches of uni-
formly thickened skin, and corns have a painful
central plug of keratin which does not contain
capillaries.
Treatment is with wart paints o r cryotherapy,
after paring down overlying keratin.
Plane warts
These are tiny, flat-topped, flesh-coloured Fig.3.8 Planewarts.
warts which usually occur on the dorsa of the
hands and the face (Fig. 3.8). They often occur
in lines due t o inoculation of the virus into Genital warts (condylomata acuminata)
scratches and abrasions. Plane warts are ex- In recent years the importance of certain types
tremely difficult t o treat effectively, and at- of genital wart viruses in the aetiology of penile
tempts at treatment may do more harm than and cervical cancer has been recognized, and
good.They will resolve spontaneously eventual- this has modified attitudes t o what was previ-
ly, and are best left alone. ously considered a minor sexually transmitted
24 Chapter 3: Bacterial and Viral Infections
Orf
O r f is caused by a parapoxvirus. It is a disease of
sheep which can be transmitted t o humans.
Those usually affected are people who bottle-
feed lambs, and butchers and abattoir workers
who handle the carcasses of sheep. The typical
clinical picture is of a solitary, inflammatory
papule which rapidly develops into a nodule of
granulation tissue-usually on a finger, but oc-
casionally on the face. The diagnosis can be
confirmed by electron microscopy of smears
from the granulation tissue. O r f lesions resolve
spontaneously in 6-8 weeks, but the disease
may act as a trigger for erythema multiforme.
cutaneous herpes simplex may also occur, and virus into a finger, painful episodes of 'herpetic
in atopic eczema this can be very extensive and whitlow' occur. The frequency of episodes of
may be life-threatening (see below). Genital herpes simplex usually gradually declines with
herpes may result from sexual transmission of advancing age.
type 2 HSV o r orogenital transmission of type I Labial herpes simplex is usually a minor cos-
HSV. metic inconvenience, and does not require
Physical contact during sport provides an- treatment. However, if episodes are frequent
other means of HSV transmission-in rugby, and troublesome, topical aciclovir may be of
herpes simplex thus acquired is known benefit. This blocks viral replication-it is not
as 'scrumpox', and in wrestling as 'herpes viricidal, and is not curative.
gladiatorum'.
Followinga primary infection the virus settles Herpes simplex and
in sensory ganglia, and may be triggered t o pro- erythema multiforme
duce recurrent lesions by a variety of stimuli. In Recurrent herpes simplex can trigger erythema
immunodeficient individuals, for example those multiforme. Prophylactic oral aciclovir may be
who are immunosuppressed following organ of considerable benefit in the management of
transplantation, o r in association with human severe cases.
immunodeficiency virus (HIV) infection, herpes
simplex infection may be clinically atypical and Eczema herpeticum (Kaposi's
run a prolonged course. uaricelliform eruption)
This is a widespread herpes simplex infection
Recurrent herpes simplex which occurs in atopic eczema. The head and
Recurrent cold sores on the lips (herpes labialis) neck are frequently affected, but lesions may
are common. Itching and discomfort in the af- spread rapidly t o involve extensive areas of skin
fected area precedes, by a few hours, the erup- (Fig. 3.1 1 ). Lymphadenopathy and constitu-
tion of a group of small vesicles. The vesicle tional upset may occur. If the disease is limited in
contents subsequently become cloudy, and distribution and the patient is seen early in its
then crusting occurs, before resolution in about course, oral aciclovir therapy is appropriate.
I 0 days.The trigger for these episodes is often However, if the lesions are extensive, and the
fever, but exposure t o strong sunlight, and patient is unwell, they should be admitted t o
menstruation are also recognized precipitants. hospital and treated with intravenous aciclovir.
Occasionally, as a result of inoculation of the If the patient is using topical steroid therapy t o
26 Chapter 3: Bacterial and Viral Infections
treat the eczema, this should be stopped until Shingles usually affects a single dermatome,
the herpes has resolved. Eczema herpeticum most commonly on the thorax or abdomen.
may recur, but in many cases subsequent The eruption may be preceded by pain in the re-
episodes tend t o be less severe. gion of the dermatome, and this occasionally
leads t o an incorrect diagnosis of internal
Herpes zoster (shingles) pathology. The lesions consist of a unilateral
Chickenpox and herpes zoster are both caused band of grouped vesicles on an erythematous
by the varicella-zoster virus. 'Shingles' is a dis- base (Fig. 3.12).The contents of the vesicles are
tortion of the Latin cingulum, meaning a girdle. intially clear, but subsequently become cloudy.
Following an attack of chickenpox the virus There may be scattered outlying vesicles on the
remains dormant in dorsal root ganglia until rest of the body, and these tend t o be more
some stimulus reactivatesit and causes shingles. numerous in the elderly. However, numerous
The middle aged and elderly are most often af- outlying vesicles (disseminated zoster) are also
fected, but it occasionally occurs in childhood. seen in immunosuppressed individuals, and
It is also more frequent in immunosuppressed their presence should prompt further investiga-
individuals. tion of the patient.After a few days the vesicles
Viral infections 27
Sacral zoster
Involvement of the sacral segments may cause
acute retention of urine in association with the
rash.
Trigeminal zoster
Herpes zoster may affect any of the divisions
of the trigeminal nerve, but the ophthalmic
division is the most frequently involved (Fig.
3.13). Ocular problems such as conjunctivitis,
keratitis andlor iridocyclitis may occur if the
nasociliary branch of the ophthalmic division is
affected (indicated by vesicles on the side of
the nose), and patients with ophthalmic zoster
should be examined by an ophthalmologist.
Involvement of the maxillary division of
the trigeminal nerve produces vesicles on the
cheek, and unilateral vesicles on the palate.
The fungi which may cause human disease in- The dermatophyte fungi are named accord-
clude the dermatophytes (Greek, meaning'skin ing t o their genus (Micmsporum, Trichophyton
plants') and the yeast-like fungus Candida albi- and Epidermophyton) and their species (e.g. M.
cans, which are responsiblefor superficial fungal canis. T. rubrum), and they can be distinguished
infections confined t o the skin, hair and nails, from one another in culture. An experienced
and mucous membranes. Other fungi can dermatologist may be able t o suggestthata cer-
invade living tissue t o cause deep infections, tain fungus is responsible for a particular case of
which may remain localized (mycetoma) o r ringworm, but the only way to establish i t s
cause systemic d~sease(e.g. histoplasmoris). identity precisely is by culture.
The dermatophytes are a group of fungi Some fungi are confined t o humans (anthro-
which are responsible for so-called 'ringworm' pophilic), others principally affect animals
infections. The vegetative phase of dermato- (zoophilic) but occasionally infect humans.
phyte fungi consists of septate hyphae which When animal fungi cause human skin lesions
form a branching network (mycelium). Candida their presence often provoles a severe inflam-
albicans is an organism composed of round o r matory reaction (e.g. cattle ringworm). Der-
oval cells which divide by budding. Apart from matophytes grow only in keratin-the stratum
its yeast form it may produce pseudohyphae corneum of the skin, hair and nails. Infection is
consisting of numerous cells in a linear arrange- usually acquired by contact with keratin debris
ment or, in certain circumstances, true reptate carrying fungal hyphae-for example, the lady
hyphae. who developed ringworm on the buttocks as
a result of her husband's habit of cutting his
toenails with his feet resting on the lavatory
Dermatophyte infections seat.
Tinea capitis
Tinea capitis is principally a disease of child-
hood, and is rare in adults.This is thought t o be
related t o a change in the fatty acid constituents
of sebum around the time of puberty. Postpu-
bertal sebum contains fungistatic fatty acids.The
principal fungi responsible for scalp ringworm
vary in different parts of the world. In the UK,
most cases of scalp ringworm are the result of
M. canis infection, usually acquired from cats,
in the USA the usual causative organism is Tri-
chophyton tonsurans, and in the Indian sub-
continent the commonest cause is Trichophyton
Fig. 4.4 Tinea on the dorsum of the hand. violaceurn.
Trichophyton violaceurn is encountered in
children of Asian families in the UK. A recent
well-defined edge (Fig. 4.4). The source of the development is the occurrence of cases of scalp
fungus is almost invariablythe patient's feet. ringworm caused by T: tonsurans in the UK.
One o r more patches of partial hair loss de-
Tinea unguium velop on an otherwise normal scalp (Fig. 4.7).
Toenail fungal dystrophy is very common in The affected scalp is scaly, and the hair is usually
adults, and is invariably associated with athlete's broken off just above the surface, producing an
foot. The involvement usually starts distally as irregular stubble. In some cases there is little
Dermatophyte infections 31
Kerion
Kerion (Greek, meaning'honeycomb') is a term
applied t o severe inflammatory scalp ringworm,
usually provoked by the fungus of cattle ring-
worm, but occasionally by other fungi. It resem-
bles a bacterial infection, with pustules and
abscesses (Fig. 4.8), and affected children are
often given repeated courses of antibiotics on
this assumption.They may also be subjected t o
surgical incision of the areas.
Cattle ringworm
In rural areas, young farm workers often suffer
from cattle ringworm -older farmers have
usually had the disease, and develop immunity
against reinfection. The face and forearms are
the areas most frequently affected. There is a
marked inflammatory reaction t o the fungus,
producing an appearance resembling a bacterial
infection (Fig. 4.9).
Children who visit farms may pick up the
fungus from gates and fences where cattle have
Fig.4.6 Tinea of a fingernail.
left keratin debris containing the organism, and
subsequently develop a kerion.
obvious inflammation, but in others this is
prominent and there is pustule formation. Tinea incognito
Occasionally the area of scalp involved is This term is applied t o a fungal infection whose
more extensive, producing an appearance sug- appearance has been altered by inappropriate
gestive of seborrhoeic dermatitis. Microsporum treatment with topical steroid preparations.
canis fluoresces yellow-green under long wave- Topical steroids suppress the inflammatory re-
length ultraviolet light (Wood's light)-see sponse t o the fungus, and the typical scaly ery-
Chapter 2. thematous margin usually disappears, leaving an
32 Chapter 4: Fungal Infections
Fig.4.10 Fungalmycelium.
34 Chapter 4: Fungal Infections
membranes by yeast-like fungi of the genus with Candida o r staphylococci, and the develop-
Candida. The commonest, Candida albicans, is a ment of prominent creases at the angles of the
normal commensalof the human digestive tract, mouth, either as a normal consequence of age,
where itexists in balance with the bacterial flora. o r in edentulous individuals who do not wear
In its commensal role, Candida is present as dentures o r who have ill-fitting dentures. Saliva
budding yeasts. In a pathogenic role, budding is drawn into the creases by capillary action, and
and mycelial forms are usually present. It only salivary enzymes macerate the skin, producing
becomespathogenicwhen situations favourable sore, moist areas.
t o its multiplication arise.These include topical In denture wearers, modification of the den-
and systemic steroid therapy, immune suppres- tures may help. If Candida is present, the topical
sion of any aetiology (e.g. lymphoma, AIDS), application of nystatin o r an imidazole antifungal
broad-spectrum antibiotic therapy, diabetes such as miconazole will also help. If staphylo-
mellitus, and the apposition of areas of skin t o cocci are isolated, topical fucidic acid may be of
produce a warm, moist environment. benefit.
The diagnosis of candidiasis can be confirmed
by culture of swabs taken from the affected Chronicparonychia
areas. This is a chronic inflammatory process affecting
the proximal nail fold and nail matrix. It occurs
Buccal mucosal candidiasis predominantly in those whose hands are
White, curd-like plaques adhere t o the buccal repeatedly immersed in water -housewives,
mucosa. If these are scraped off, the underlying barstaff, florists, fishmongers. A more wide-
epithelium is inflamed and friable. I t may be spread use of mechanical dishwashers, rather
treated with nystatin pastilles o r oral suspen- than the human variety, may be associated with
sion, amphotericin lozenges, miconazole oral a decline in the incidence of this disorder.
gel o r itraconazole liquid. The clinical appearance is of thickening and
erythema of the proximal nail fold ('bolstering'),
Angular cheilitis (perleehe) and loss of the cuticle (Fig. 4.1 2). There is often
Angular cheilitis is an inflammatory process an associated nail dystrophy. Candida albicans
occurring at the corners of the mouth (Fig. plays a pathogenic role, but bacteria may also be
4.1 I). involved. The absence of the cuticle allows ac-
The main factors involved in its causation, ei- cess of irritant substances, such as detergents,
ther alone o r in combination, include infection t o the area beneath the proximal nail fold, and
Intertrigo
'Intertrigo' is a term applied t o maceration
which occurs where rwo skin surfaces are in ap-
position-groins.axillae,submammary regions.
o r beneath an abdominal fat apron. Obesity and
poor hygiene are contributory factors. Candida
superinfection is often present, and is suggested
clinically by the presence of creamy 'satellite'
pustulesatrhe marginsofthe affected areas.The
pustules are easily ruptured, leavinga collarette
of scale, and this gives a characteristic scalloped
edge t o the area of intertrigo.
Fig.4.12 Chronicparonychia Therapy with a combined topical prepara-
tion containing an anti-Candida agent; such
as miconazole, and hydrocortisone is usually
this probably contributes t o the inflammatory beneficial, but attention to hygiene is also
process. important.
This condition is quite distinct from acute
bacterial paronychia, in which there is a short
history, severe discomfort and ample produc- Pityriasis versicolor
tion of green pus. Pressure on the swollen nail
fold in chronic paronychia may produce a tiny This condition occurs predominantly in young
bead of creamy pus from underthe nail fold, but adults, and is caused byMalassezia yeasts, which
that is all. are normal skin commensals present in pilose-
Treatment consists of advice t o keep the baceour follicles. It is a common disorder in
hands as dry as possible by wearingcotton-lined temperate zones and is seen even more fre-
rubber o r PVC gloves when worldng, and quently in tropical climates. The reason why
topical anti-Candida therapy, for example with these yeasts multiply and produce skin lesions in
an imidawle. certain individuals is unknown.
On a fair skin, the lesions are light-brown
Balanitis/uulvouaginitis macules with afine surfacescale, and they occur
Small white patches orerodedareasare present predominantly on the trunk and upper arms
on the foreskin and glans o f the uncircumcised (Fig. 4.13). They are usually asymptomatic, but
penis. Predisposing factors are poor penile are a cosmetic nuisance. O n pigmenred s l h ,
hygiene, and diabetes mellitus. Candida balanitis the typical appearance is of patchy hypopigmen-
may be a recurrent problem if a sexual partner ration.The loss of pigment is thought t o be re-
has Candida vaginitis. lated t o production of azelaic acid by the yeasts
Candidavulvovaginitis presents with acreamy which inhibitstyrosinaseand thereby interferes
vaginal discharge and itchy erythema of the with melanin production. The reason for the
vulva. Pregnancy, oral contraceptives and dia- brown colour on pale skin is unknown. The
36 Chapter 4: Fungal Infections
colour variation, depending on the background few minutes during bathing. This will usually
skin colour, is the reason for the 'versicolor' in clear the organism in 2-3 weeks.Topical imida-
the name. zole antifungal creams and ketoconazole sham-
The diagnosis can be confirmed by micro- poo are also effective against Malassezia, as is
scopic examination of skin scraping in a topical terbinafine. Oral itraconazole is an alter-
mixture of 10% potassium hydroxide and native (200 mg daily for 7 days). Griseofulvin and
Parker Quink ink (Fig. 4.14), when character- oral terbinafine are ineffective.
istic clumps of round spores and short, stubby Pityriasisversicolor tends t o recur, and treat-
hyphae can be seen (an appearance which has ment may have t o be repeated. Hypopigmented
been called'spaghetti and meat balls'). areas may take a considerable time t o repig-
A simple treatment is selenium sulfide, in the ment, and their persistence should not be taken
form of a shampoo (Selsun), left on the skin for a as evidence of treatment failure.
Ectoparasite Infections
Scabies,37 Pedicuiosis.41
Crusted(Narwegian)scabies,40
inflammatory papules which occur mainly must be found, and this usually requires some
around the axillae and umbilicus, and on the expertise. Look carefully, in good light, at the
thighs (Fig. 5.2).The rash is an allergic reaction hands and feet. A magnifying glass may be of
t o the mites. some help, but myopia is a distinct advantage.
In addition t o these primary skin lesions, Once a burrow, or a suspected burrow, has
there may be secondary changes such as been identified, i t should be gently scraped off
excoriations, eczematization and secondary the skin with the edge of a scalpel blade-
bacterial infection. In some parts of the world, dermatologists sometimes use a blunt scalpel
secondary infection of scabies lesions with known as a 'banana' scalpel for this task (see
nephritogenic streptococci is associated Chapter 2).The scrapings should be'placedon a
with the development of poststreptococcal microscopeslide with afew drops of 10%potas-
glomerulonephritis. sium hydroxide, covered with a coverslip, and
examined under the microscope.The presence
Diagnosis of mites, eggs, or even egg-shells, confirms the
Absolute confirmation of the diagnosis can only diagnosis (Fig. 5.3). Do not attempt t o scrape
be made by demonstrating the mites or eggs any lesions on the penis-the proximity of a
microscopically. In order t o do this, burrows scalpel t o this area leads t o understandable
Scabies 39
apprehension,and is in any case rarely rewarded (crotamiton 10% and hydrocortisone 0.25%)
by the demonstration of mites. can be used on residual itchy areas. I t is not
An alternative t o the scalpel routine is what necessary t o 'disinfest' clothing and soft
might be referred t o as the'winkle-picker' tech- furnishings-laundering of underwear and
nique. If the vesicle at the end of a burrow is nightclothes is all that is required.
opened with a needle, the tip of which is gently
moved around within it, the mite can often be Available treatments
removed, with a theatrical flourish, on the end Malathion 0.5%
of the needle. Aqueous preparations are preferred because
they do not irritate excoriated o r eczematized
Treatment skin. Wash off after 24 h.
Scabies is treated by eatingyoung alligators
and washing the skin with urine. (Mexican Folk 5%Permethrin cream
Medicine) Wash off after 8- 1 2 h.
It is important t o explain t o patients precisely
how t o use their treatment, and written ex- A single application of malathion o r permethrin
planatory treatment sheets are useful.All family is often effective, but a second application 7 days
members, and close physical contacts of an later is recommended.
affected individual, should be treated simul-
taneously. Topical agents should be applied Benzyl benzoate emulsion
from the neck t o the toes, and patients should Three applications in a 24-h period. O n the
be reminded not t o wash their hands after ap- evening of day I apply the emulsion from the
plying treatment. In infants, the elderly and the neck t o the toes.Allow t o dry, then apply a sec-
immunocompromised, in whom burrows can ond coat. The following morning apply a third
occur on the head and neck, it may be necessary coat, and then wash off the benzyl benzoate on
t o extend application t o these areas. Itching the evening of day 2. Treatment is then com-
does not resolve immediately following treat- plete, and this should be stressed t o the patient
ment, but will improve gradually over 2-3 because repeated use will produce an irritant
weeks as the superficial epidermis, contain- dermatitis.
ing the allergenic mites, is shed. A topical anti- Benzyl benzoate is a very effective scabicide,
pruritic such as Eurax-Hydrocortisone cream but it is irritant, and i t has been superseded by
40 Chapter 5: Ectoparasite Infections
more modern preparations in the UK. How- o r paresis. In these circumstances, when the
ever, because it is inexpensive it is still used in host does not itch o r cannot scratch, burrows
many parts of the world. are n o t destroyed and the mite population mul-
tiplies unchecked. Crusted scabies also occurs
neatment of infants more frequently in individuals with Down's
As burrows can occur on the head and neck it syndrome.
may be necessary t o extend application of topi- The crusted skin lesions can contain millions
cal agents to these areas. Malathion prepara- of mites and eggs, and these are shed into the
tions are not recommended for children under environment on flakes o f 1keratin.Anyone com-
6 months and permethrin is not recommended ing into contact with a patient suffering from
in infants younger than 2 months. crusted scabies is at risk of acquiring ordinary
Because of the availability of non-irritant scabies, and undiagnosed cases may be respon-
agents, benzyl benzoate is not recommended sible for outbreaks of scabies in hospitals and
for use in infants, but if it is employed it should residential homes.
be diluted to reduce i t s irritancy.
Clinical features
Weatment in pregnancy The hands and feet are usually encased in a
There is understandable concern about poten- thick, fissured crust and areas of crusting may
tial toxic effects on the fetus of scabicides when be present on other parts of the body, including
used in pregnancy. However, there is no defini- the head and neck.The nails are often grossly
tive evidencethatany ofthe currently employed thickened (Fig. 5.4).The changes may resemble
topical scabicides has been responsible for psoriatic scaling o r hyperkeratotic eczema.
harmful effects in pregnancy following appro- and this can lead t o the diagnosis being missed.
priate use. Hence, in the absence of evidence Burrowrare usually impossible t o identify in the
offetal toxicity, use of malathion or permethrin crusted areas, but may befound on less severely
appears t o be safe. affected parts of the body Microscopy of the
scales reveals numerous mites and eggs.
Fig.5.4 Crustedscabiesin a
youth with Dawn's syndrome.
large head louse populations, secondary skin Clothing lice (Pediculus humanus)
sepsis and probably also anaemia, and were The louse
chronically unwell as a result. Has very little 'nous',
Its only pursuit
Tveatment Is the hirsute.
The approach t o treatment has changed in re- (I. Kenvyn Evans)
cent years, and it is now considered appropriate The clothing o r body louse is a parasite which
t o employ a strategy that includes both physical thrives in associationwith poverty and poor hy-
and chemical methods. Chemical means of con- giene. It lives on, and lays its eggs in the seams of,
trol, employing insecticides, have been widely clothing, and only moves onto the body t o feed
used throughout the world. Insecticides are on blood. It is still common in the poorer coun-
easy t o use and convenient, and they have tries of the world, but in an affluent society its
proved very effective. However, there is con- usual hosts are tramps and down-and-outs who
cern about potential adverse effects, particu- have only one set of clothes which are never re-
larly from residual insecticides such as lindane moved o r cleaned. An individual who regularly
(which is no longer used in some parts of changes clothing and maintains a reasonable
the world, including the UK), not only on standard of hygiene will never harbour clothing
humans but also on the environment. In addi- lice because the lice will not survive laundering
tion, there is evidence of widespread resis- and ironing of garments. Clothing lice are vec-
tance of head lice t o malathion and pyrethroid tors of epidemic typhus, a rickettsia1 disease
insecticides. which has been responsible for millions of
deaths over the centuries.
Clinicalfeatures
- Malathion.
Clothing lice usually provoke itching, and their
host is often covered in excoriations.
-
* Carbaryl.
Syntheticpyethroids:
pemethrin
The itching appears t o be the result of an
acquired hypersensitivity t o louse salivary anti-
gens. If clothing louse infection is suspected
phenothrin.
there is no point in searching the patient for
lice-you may be lucky and find an occasional
louse at lunch on the body, but it is the clothing
Preparations with an aqueous basis are you should examine (Fig. 5.8).
preferable t o alcoholic solutions because they
are less likely t o irritate an excoriated scalp, Tveatrnent
do not irritate the bronchi of asthmatics, and All the patient requires is a bath. A complete
are not flammable. None of these insecticides change of clean clothing should be supplied
is fully ovicidal, and treatment should there- and the infested clothing either destroyed o r
fore be repeated after 7-1 0 days in order t o laundered at temperatures of 60C o r above.
kill any louse nymphs emerging from surviving D r y cleaning o r use of a tumble drier are
eggs. alternatives.
A simple physical method of treatment in-
volves washing the hair with an ordinary sham- Crab lice (Pthirus pubis)
poo followed by the application of generous It's no good standing on the seat
quantities of conditioner. The hair is then The crabs in here canjump I 0 feet.
combed with a fine-toothed comb with closely lfyou think that's rather high,
set teeth, which removes any lice.This process Go next door, the buggers fly!
is repeated every 4 days for 2 weeks. (Toilet graffito)
44 Chapter 5: Ectoparasite Infections
Crab lice, also known as pubic lice and love bugs, crab's pincers (Fig. 5.9),with which itgrasps hair.
and in France as papillonsd'amour, are usually Female crab lice, like head lice, stick their eggs
transmitted during close physical contact with t o hair shafts with a cement material.
an infected individual, in spite of the above alle-
gation of contagion from hinged lavatory com- Clinicalfeatures
ponents. A t one time they were thought t o be Itching, usually nocturnal, is the symptom which
rather sedentary lice, but experiments subse- draws the host's attention t o these little passen-
quently demonstrated that when its host is gers. Self-examination then reveals the reason
sleeping the crab louse becomes quite active. It for the itch, and the doctor is often presented
is a louse adapted t o living in hair of a particular with a folded piece of paper containing 'speci-
density. It cannot colonize scalp hair, except at mens'. When folded paper is opened it has a
the margins of the scalp, but pubic, axillary, tendency t o flick its contents in all directions,
beard and eyelash hair are perfectly acceptable leaving the unlucky recipient anxiously awaiting
t o it, and in an extremely hairy male most of the signs of personal contamination for weeks
body may resemble a crab louse adventure play- thereafter.
ground.Thecrab louse is so named because of Lice are usually visible on the affected areas,
its squat shape and powerful claws, resemblinga but sometimes their eggs, which are a brown
Papular urticaria 45
neatment
Malathion and carbaryl are effectiveagainstcrab
lice. Aqueous preparations should be used be-
cause alcohol basis preparations will irritate the
scrotum. The whole body should be treated.
including the scalp if there is evidence of lice on
the scalp margins. Sexual contacts should also
be treated.The treatment should be repeated
after an interval of7-IOdays.
Eyelash infection may be treated by the appli-
cation of white soft paraftin (Vaseline) three
times a day for 2-3 weelcThis acts by blocking
the louse respiratory system, thereby ruffo- Fig.5.11 Papularurticaria.
cating the insects.
coriated.They develop as a result of a hypersen- numbers are small in comparison with the fleas
sitivity response t o antigens in the arthropods' in various stages of development scattered
saliva. Eventually, in many people, immunologi- throughout the household. Flea eggs are not
cal tolerance t o the antigens develops and they sticky, and when laid by fleas feeding on an ani-
subsequently do not react t o the bites. mal they drop out ofthe coat into the surround-
ings -the cat-basket, the carpet, the sofa o r the
Fleas counterpane. Eggs, larvae, pupae and adult fleas
May the fleas of a thousand camels infest your are present in all these areas. Hence, the house
armpits! (Arab curse) should be treated, as well as the pets. A prepa-
The commonest cause of papular urticaria ac- ration designed t o be sprayed around the house
quired in the home environment is flea bites. It on carpets and soft furnishings is a combination
is not the human flea, Pulex irritans, which is of the insecticide permethrin with methoprene,
responsible, but fleas whose natural hosts are a synthetic equivalent of an insect growth regu-
household pets. A familiar clinical picture is of latory hormone, in an aerosol can (Acclaim
multiple lesions, some of which may form blis- 2000).The permethrin kills adult fleas, and the
ters, around the ankles of women (Fig. 5.12). methoprene blocks the metamorphosis of flea
Men are rarely affected, because socks and larvae into adults. I t should be sprayed on car-
trousers deny fleas access t o the ankles. pets and soft furnishings, and the animals' sleep-
Cats and dogs are perambulating quadripedal ing areas, and will confer protection against flea
'meals-on-wheels' for the fleas. However, al- infestation for4 months. Another useful antiflea
though fleas are present on the animals their agent is lufenuron (Program), which is given
orally t o the animal, is ingested by feeding fleas,
and interferes with the production of chitin by
flea larvae, thereby preventing their further
development.
Occasionally, bird fleas will gain access t o
homes from nests under the eaves, and may be
responsible for more extensive lesions of papu-
lar urticaria.
t o the bites of the bugs usually results in papular combings from the animal's coat and demon-
urticaria o r bullous lesions. These insects are strating the mite microscopically. Once the
5-6mm long, dark brown in colour, and can animal has been treated by a veterinary
move quite rapidly. Fortunately, bed-bug infes- practitioner the human skin lesions resolve
tation of houses in developed countries is now spontaneously.
uncommon, but if i t is suspected the local Bird mites may gain access t o houses from
environmental health department should be nests under the eaves, and can cause itchy papu-
asked t o inspect and disinfest the property. lar lesions.
Acne vulgaris
Introduction About 80% of people develop some spottiness.
Acne may be very mild indeed but at its most
This chapter deals with disorders which cause severe, gross and unsightly changes are seen.
papules and pustules, often lknown in the ver- Acne may be associated with underlying en-
nacular as 'spots' o r 'zirs'. Some are aetiologi- docrinological abnormalities (see below) but
caliy related and can properly be called variants usually it is n o t
of acne (a corruption of the Greek okme-a
point). Others produce lesions closely o r su-
Age of onset and course
perficially resembling'true' acne: the acneiform The first problems are usually encountered in
disorders and rosacea. A summary of the'acne adolescence, although there are exceptions
(see below).
family'isgiven below.
Lesions of acne vary considerably with time.
Most patients notice marked fluctuations in the
number and severity of spots, and in girls this is
often related t o the menstrual cycle.The con-
Acnedgaris dition frequently deteriorates attimes of stress.
- 'Classical'
Infantile andjuvenile onset
* Lateonset
Acne usually gets worse for a while before
gradually settlingafter2-3 years, and disappear-
-
ing altogether in the majority. The peak of
* Severe (acneconglobata:nodulocystic)
With systemic symptoms (acnefulminans) severity is earlier in girls than boys. In some
individuals the time-course may be much more
Semndqacne prolonged, with lesions continuing t o develop
-.
* Endocrine associated
Medicaments
oils
well into adult life.
There are two groups, described below, in
whom true acne develops outside adolescence.
* Chloracne
i Infantileljuvenile acne
Typical acne is occasionally seen in infants and
Clinicalfeatures 49
Late-onset acne
Some women develop acne in their twenties
and beyond, often with marl<ed premenstrual
exacerbations. Endocrinological investigation is
generally unrewarding, but some have polycys-
ticovary syndrome (see p. 54).
- Face,anypmofwhichmaybeinvolved
* Neck,esp&allypostenorly
The individual lesions of acne
- Upper back
AnteriarcheJt,inaninvertedv'fmm the
shoulderstothexiphisternum
Acardinaifeature is thatthereareseverai differ-
enttypes of lesion at any one time.
* Shoulders
* Ears
50 Chapter 6: Acne, Acneiform Eruptions and Rosacea
- open (WaCkheads)
Papules
PUSNleS
may itch o r be quite painful. Papules develop
rapidly, often over a few hours, and frequently
become pustular as they evolve. They resolve
-
* Nodules
cys=
* Scars
over the course of a few days. N e w lesions may
arise in exactly the same site on many occasions.
Systemic symptoms
Pathogenesisof acne
(acne fulminans)
Very occasionally a young man (almost always)
The aetio-pathology of acne remains t o be elu-
develops severe nodulocystic acne accompan-
cidated fully. However, several key features may
ied by fever, malaise and joint pain and swelling.
contribute t o the final picture (see Fig. 6.6), al-
This is known as'acnefulminans'.
though this does not fully explain every aspect
of the disorder: the occurrence of prepubertal
acne, for example.
As the inflammation subsides, a variable
amount of fibrosis occurs. This may produce
scarring, particularly if repeated episodes occur
in the same site. Sometimes epithelia1remnants
become walled off by fibrosis, producingcysu.
Systemic therapies
Treatment of acne
Antibiotics are the mainstay of the treatment of
papulopustular acne. It is not known precisely
Topical therapies
how they w o r k but they reduce bacterial
Many over-the-counter remedies ~ a l yon sulfur
counts, at least initially, and may also have direct
and other astringents which mal<ethe skn flalcy
anti-inflammatory effects.
and unblock hairfollicles.
The most effective are the tetracyclines and
Topical antiseptics such as povidone iodine
erythromycin.To work, antibiotics must be fat
and chlorhexidine are often PI-escribed,but are
soluble, and the penicillins are therefore useless.
of little proven value.
Mosttetncyclinesshould betaken on an empty
Benzoyl peroxide is widely used. It reduces
stomach. Tetracyclines are contraindicated in
comedones (it is 'comedolytic') but must be
the under twelves, and in pregnant o r lactating
used regularly and in the long term.There are
women.
severalstrengths:startwithaweakprepaation.
Cyproterone acetate is an antiandrogen
applied once daily, and gradually progress to
which can only be given t o women. I t is given
higher concentrations.
with oestrogen t o prevent menorrhagia and t o
Vitamin A derivatives (retinoids) and
ensure contraceptive cover (it will feminize a
retinoid-lilce agents also have comedolytic
male fetus). Its effect is rather slow.
activity. Preparations in this category include
13-cis-retinoic acid (isotretinoin) is a highly
retinoic acid, isotretinoin (13-cis-retinoic
effective oral vitamin A derivative which dra-
acid) and adapalene. All work well but can be
matically reduces sebum production. It has
irritant.
several side-effects: dry lips,eyesand skin, nose-
Topical tetracyclines, erythromycin and clin-
bleeds. mild alopecia. aches and pains. It also
damycin are available, and are generally applied
raises blood fat levels and may affect liver
once daily.All have been shown t o be useful in
function tests. The most serious problem is
milder acne.There are some preparations that
teratogenicity. Female patients must not be-
combine antibiotics with other agents such as
come pregnant when taking isotretinoin, as it
benzoyl peroxide.
will produce fetal abnormalities. lsotretinoin is
only available on hospital prescription in the
UK. Over 90% of patients have complete clear-
ance of their acne and in up t o 80% there is no
relapse.
Topical Steroids can be used intralesionally o r
Benzoylperoxide systemically in severe acne (they are virtually
Retinoidsand retinoid-likeagents always needed in acnefulminans).
* Sulfurandarningents
Topical antiseptics.antibiotiicsand Surgical intervention
combinationproducts
Simple measures, such as removing multiple
S p a n i C comedones with a comedone extractor, may
-
* Antibiotics
Cvpmteroneacetate
* 13-cis-retinoicacid (isouetinoin)
improve the appearance. It certainly gives great
pleasure and satisfaction t o a girl- o r boyfriend
who likes t o pop out blackheads! Large. residual
Steroids cysts may need t o be excised, but there is a risk
of keloid scarring. Plastic surgeons can some-
sugicalinnlmllim
times help acne scarring by dermabrasion, but
this must not be attempted until the acne is fully
under controL
54 Chapter 6: Acne. Acneiform Eruptions and Rosacea
-
* Acneisdue to'hormonalimbalance'
Acne~relatedtosexualbehaviour
help overcome the devastation wreal<edby this
degree of acne.
Allmbbish!
- Hormones arenormalinthevastmajority
Neithertooliftle,nortoomuchsexmakes
anvdifference ithank&ness!l
Modamre
Morepapulopusrularlesionson the faceor
werawiderarea rmdlw occsaionalnodules
scrrrr
Assessment of the patient Vezywidvad papulopuetularlesions
I t can be useful t o consider acne in three broad andlorncdulocyrticlesions rmdlorsptemic
severity bands: mild, moderate and severe. BYmPtOma
Mild ocne may respond r o topical treatment oracneofmoderateseverity,failjngtosetUe
alone. Begin with benzoyl peroxide, retinoic within 6 months of therapy
or acne ofwhateverseverityeth significant
acid, isotretlnoin or adapalene, andtor a topical
psychologi~upae~
antibiotic.An antibioticlbenzoyl peroxide com-
bination can be a useful option.
Moderateacne should initially be treated with
a combination of a topical agent and oral oxyte- Secondary acne
tracycline o r erythromycin in a dose of 500mg
twice daily. Continue forat least 3 4 months be- Acne lesions may arise as a consequence of
fore reassessing. Alternative tetracyclines have other primary pathological processes. Such
their advocates: some may be better absorbed 'secondary' acne is often monomorphic and
o r tolerated, but most are more expensive and generally mild.
there is generally no indication for their use as An exception is acne occurring in patients
first-line agents. with endocrine abnormo1ities.The commonest is
If the response is not satisfactory, the acne polycystic ovary syndrome, in which acneofany
should be managed as outlined below. severity may accompany hirsutism, menstrual
Severeocne may becontrolledt o someextent irregularities and infertility.Any cause ofabnor-
by systemic antibiotics, but this degree of acne mally high circulating androgen levels (such as
often demands more aggressive treatment. tumours) may also cause quite severe acne.
Girls may respond t o cyproterone acetate with while lesions in Gushing's syndromeare milder.
Acneiform disorders 55
keloids develop. The process may involve the Fig. 6.10 Rhinophyma.
nape of the neck, when it is usually termed acne
keloidalis.Treatment is unsatisfactory.
Acne excoriee (desjeunes filles) is typically seen
in teenage girls who present with facial excoria- Rosacea is an important differential diagnosis
tions but very few primary lesions.There are no of acne, and is sometimes called 'acne rosacea'.
comedones.This is not true acne but a form of I t most frequently affects middle-aged women,
neurotic excoriation (see Chapter 20),and pa- but it may occur in men, and can occur at any
tients need t o be given a clear explanation.Tran- age. The sites of predilection are the central
quillizers may help. cheeks, forehead and glabellar region, end of
Pityrosporurn folliculitis causes follicular the nose and chin (Fig. 6.9). The eruption
papules and pustules on the trunk, without consists of small papules and pustules arising
other features of acne.The condition responds in crops on an erythematous, telangiectatic
t o antifungal agents such as miconazole. background.
In keratosis pilaris small spiky projections de- There are no comedones. Patientsfrequently
velop at the mouth of hair follicles, especially on complain that their face flushes easily with heat
the upper, outer arms and shoulders (Fig. 6.8). o r alcohol, and migraines are more common. In
Lesions may appear on the face, especially in men, severe involvement of the nose leads t o
children, and are occasionally pustular. A family marked sebaceous hyperplasia known as rhino-
history is common.Topical retinoic acid may be phyma (Fig. 6.10).
helpful. The treatment of choice is tetracyclines,
Acneiform disorders 57
given for several weeks in similar doses t o those Perioral dermatitis (note for strict classical
for moderate acne (see above). Topical metro- scholars: it should really be 'circum-oral') pro-
nidazole may help. It may be possible t o tail off duces a clinical appearance somewhat reminis-
the treatment, but the condition often recurs. cent of rosacea (see Fig. 22.2), and is often
Topical steroids make matters worse. Rhino- associated with topical steroid abuse. (For
phyma is best dealt with by plastic surgeons. more details see Chapter 22.)
Eczema
To keep three or four spats ofeczemo in o sionally, mostofthe body surface is affected, and
private port ofmy body and now and then to eczema is one cause of generalized exfoliative
scald or bathe them wikh hot water behind dermatitis.
closed doors. Ah,is this not happiness? Other changes in the skin which may
(Tim Shangt'an) accompany eczema include scratch marks
The terms eczema (Greek, meaning 'to boil and secondary bacterial infection. Prolonged
over') and dermatitis are synonymous.'Atopic scratching and rubbing the sldn tends t o polish
eczema' is therefore the same as 'atopic fingernails, and patients with chronic eczema
dermatitis' and 'seborrhoeic eczema' and often have nails which lookas if they havea coat
'seborrhoeic dermatitis'are the same. Eczema/ ofclear nail varnish.
dermatitis is a type of inflammatory reaction
pattern in the sldn which may be provoked by a
number of externai or internal factors. Classification
58
Exogenous eczema 59
1
1
--
Exogenm
I
* ~malyirritantmntactdennatitir
* Allergiccontactdermatilis
-
a AtopicMema
Sebrrhwicdennatitis
] - Discoid eczema
Varicoseeaema
.
'
Endogenousenernaofthepalmrandroles
j fiteatati'cenema (enemamquele)
-I
Exogenous eczema
more recent resurgence of interest in the sus- problems from watches can usually be avoided
pender belt has not been accompanied by skin by wearing a'swatch' watch, as the only metal in
problems, because the clips are coated metal o r contact with the skin is the stainless steel bat-
synthetic material. I t is the jeans stud which has tery compartment.
become an important source of nickel, and
a patch of eczema adjacent t o the umbilicus is Colophony
virtually pathognomonic of nickel sensitivity This is a resin which is a component of some ad-
(Fig. 7.4). hesive plasters.
If nickel dermatitis is suspected, look at the
skin on the earlobes and wrists. In spite of being Rubber dermatitis
aware that costume jewellery provokes a skin During the manufacture of rubber, chemicals
reaction, many women continue t o wear a are used t o speed up the vulcanization process
favourite pair of earrings from time t o time, and (accelerators) and t o prevent its oxidation (an-
will have dermatitis on the ears. Nickel dermati- tioxidants), and these can cause allergic contact
tis on the wrists is usually caused by the metal dermatitis.
buckle on a watch-strap. Stainless steel in wrist- In recent years there has been a marked
watches does not cause any problems because increase in the occurrence of reactions t o
although steel contains nickel it is tightly bound natural rubber latex protein in latex gloves.
and does not leach out. Latex protein can provoke an immediate hyper-
The multitude of folk who have adopted the sensitivity response, and reactions range from
'fashion' of having their delicate bodily parts contact urticaria t o rhinitis, asthma and anaphy-
pierced are perhaps fortunate in that the vari- laxis. Individualsaffected are most often health-
ous rings and barbells dangling and protruding care workers who frequently wear latex gloves,
from them are made of steel, and do not pro- but patients who have undergone multiple
voke dermatitis unless further embellished with procedures, most notably persons suffering
costume jewellery. Anyone who is allergic t o from spina bifida, may also be affected. A signifi-
nickel should be advised t o avoid costume jew- cant factor contributingt o this problem appears
ellery (unless it is known t o be nickel free), bare t o have been that the demand for latex gloves
metal clips on underwear, metal buckles on outstripped supply, and some manufacturers
shoes and metal zips. The metal stud on the subsequently produced gloves containing large
front of jeans can be replaced by a button, and amounts of free latex protein.
62 Chapter 7: Eczema
involvement presents as itchy, diffuse scaling Mild o r moderate potency topical steroids
on an erythematous background. O n the face, will usually suppress the eczema.
there is scaly erythema in the nasolabial folds
and on the forehead, eyebrows and beard area Endogenous eczema of the palms
(Fig. 7.6). Lesions on the chest are often mar- and soles
ginated. Flexural involvement produces a moist, Some patients develop a symmetrical pattern
glazed erythema. Particularly severe sebor- of eczema affecting the palms and soles which is
rhoeic dermatitis occurs in patients suffering
from AIDS.
Seborrhoeic dermatitis usually requires
treatment over many years, as there is no cure
for this condition. I t is important t o make this
clear t o patients, who otherwise tend t o t r y
many treatments in their quest for a permanent
solution t o the problem.Topical hydrocortisone
is effective, but the problem recurs when treat-
ment is stopped. Steroid lotions o r gels and tar
shampoos will help the scalp.
Ketoconazole shampoo and cream, and imi-
dazole/hydrocortisone combinations are also
effective.
Discoid eczema
In this disorder, scattered, well-demarcated
areas of exuding and crusting eczema develop
on the trunk and 1imbs.A potent topical steroid
is usually required t o keep the condition con-
trolled. Its aetiology is unknown.
Varicose eczema
Chronic venous hypertension is frequently as-
sociated with eczematous changes on the legs.
Secondary spread t o the forearms may occur. Fig. 7.6 Facial seborrhoeic dermatitis.
Asteatotic eczema
W i t h increasing age, the lipid content of the
stratum corneum decreases, and elderly skin is
particularly susceptible t o 'degreasing' agents.
Asteatotic eczema (also known as eczema
craquele) is usually seen on the legs, but it may
also occur on the lower abdomen and arms, and
occasionally it is generalized. It is common in el-
derly patients admitted t o hospital and bathed
more frequently than they bathe at home. A
crazy-paving pattern develops (Fig. 7.8). and the
skin itches. Treatment with an emollient is
sometimes adequate, but a mild topical steroid
ointment is often necessary.
Fig.8.1 Schematic
representation of a psoriatic
plaque.
distinguish scalp psoriasis from severe sebor- thick and sticks in large chunks t o bundles of
rhoeic dermatitis (see also flexural psoriasis hair. This is known as 'pityriasis amiantacea'.
below), but psoriasis is generally thicker. As a There may be temporary hair loss in severe
rule of thumb, if you can feel scalp lesions as well scalp psoriasis.
as see them, they are probably psoriasis.
Lesions vary from one o r t w o plaques t o a Nail psoriasis
sheet of thick scale covering the whole scalp Nail abnormalities are frequent, and are impor-
surface (Fig. 8.3). Rarely,the scale becomes very tant diagnostic clues if skin lesions are few, o r
70 Chapter 8: Psoriasis
G u t t a t e psoriasis
Guttate psoriasis often develops suddenly, and
may follow an infection, especially a streptococ-
cal sore throat. I t is a common way for psoriasis Guttatepsoriasis.
t o present, particularly in young adults.
Gutta is the Latin for 'drop'. Most lesions are totic scale in psoriasis, and the shape of the le-
about a centimetre in diameter (Fig. 8.6), and sions (round in guttate psoriasis; oval in pityria-
usually paler in colour than established plaque sis rosea). Guttate psoriasis may itch.
psoriasis, at least initially. The main differential The lesions of guttate psoriasis often resolve
diagnosis is pityriasis rosea (see Chapter 15), rapidly, but in some patients the patches enlarge
best distinguished by the presence of parakera- and become stable plaques.
Clinical patterns of psoriasis 71
Flexural psoriasis
Flexural involvement in psoriasis may accom-
pany typical plaque lesions, but is also common-
ly seen alone, o r associated with scalp and nail
changes. Lesions may occur in the groin, natal
cleft, axillae, umbilicus and submammary folds.
Maceration inevitably ,occurs, and the surface
scale is often lost, leaving a rather beefy erythe-
matous appearance (Fig. 8.7). It may be difficult
t o distinguish this from ftexural seborrhoeic
dermatitis, so look for nail changes o r evidence
o f psoriasis elsewhere. Some dermatologists
believe in an overlap state between the two, and
call such changes'sebo-psoriasis'.
Flexural psoriasis is often itchy.Watch out for
a secondary contact sensitivity from the use o f
proprietary anti-itch preparations.
Brittle psoriasis
Occasionally you will see patients whose psori-
asis does n o t consist ofthick, stable plaques, but
o f thin, irritable scaly areas (Fig. 8.8). Lesions
may arise de nova o r develop suddenly in a pa- Fig. 8.8 Widespread 'brittle' psoriasis.
tient whose psoriasis has been stable for years.
One reason for this is systemic steroid therapy
(often for another condition), and potent topi-
cal steroids can also induce stable psoriasis t o Chapter 15) o r even acute pustular psoriasis
become 'brittle'. (see below).
The significance o f brittle psoriasis is that the
lesions may rapidly generalize, especially if Erythrodermic psoriasis
treated with potent agents (see treatment When psoriatic plaques merge t o involve most,
section below), leading t o erythroderma (see o r all, o f the skin a state o f erythroderma o r
72 Chapter 8: Psoriasis
Tar
Tar has been used for many years, particularly in
combination with UV radiation.The most effec-
* Salicylicaad tive preparations are extracts of crude coal tar.
Topicalsteroids Attempts have been made t o refine tar t o make
-
* Dithrand(anthralin)
Vitamin D analogues(e.g. calciporriol,
tacalcitol)
it more cosmetically acceptable, but the most
effective forms still seem t o be the darkest,
smelliest and messiest. Consequently, not many
* VitaminAanalogues patients will use tar forwidespread, routine use.
1 Ultravioletradiation
However, in bath oils o r in ointment mixtures
tar may be helpful, and is very valuable in scalp
spmnic
PWA (pMralen+ultravioletA) disease.
Retinoids
* Cytotoxics.e.g.methomxate,azathioprine. Topical steroids
hydmxycarbamide(hydmxyurea) Topical steroids do not eradicate psoriasis, but
* Systemicsteroids
may suppress it. Some dermatologists say they
Ciclorporin(cyclosporin)
never use topical steroids in psoriasis because
of the risks (they may induce'brittle' psoriasis).
However, if used with care in stable disease, and
It is an old adage that if there are many treat- on the scalp and in the flexures, they can be
ments for a disease, none works perfectly. useful.
This is certainly true of psoriasis.Although each
modality is useful in some patients, all represent Dithranol (anthralin)
a degree of compromise in terms of safety, ef- Dithranol can convert psoriatic plaques into
fectiveness o r convenience. Many patients re- completely normal-looking skin. The mode of
quire a regimen of different agents for different action is unknown. The 'Ingram regime'-
sites at different times. a combination of dithranol, tar and UV radia-
tion -has been used for years: most patients
Topical therapies can be cleared in about 3 weeks of daily treat-
Many agents can be used topically t o induce a re- ment. Originally, the dithranol was left on the
mission o r an improvement. Most are safe, but skin for 24 h, but 'short-contact' therapy is just
they are tedious for patients t o use, as treat- as good.
ment may have t o continue for months o r even Dithranol seems t o work best in Lassar's
indefinitely. paste (starch, zinc oxide and salicylic acid in
white soft paraffin), but is also available in
EmoIlients cream and ointment bases. Always begin with
Some patients are prepared t o tolerate plaques a low concentration (0.1%) and increase as
(especially on covered sites) if scaling can be necessary.
controlled. Emollients such as white o r yellow The main complications are staining (due
soft paraffin o r lanolin may accomplish this. t o oxidation t o a dye) and burning. Skin staining
is temporary, but baths, bedding and clothes
Salicylic acid may be permanently marked. Dithranol burns
Salicylic acid is a 'keratolytic' agent and helps can be very unpleasant, especially around the
t o reduce scaling. It can be used with tar in eyes. Patients must be taught t o use dithranol
mixtures, and is also combined with a potent carefully.
74 Chapter 8: Psoriasis
Vitamin D and vitamin A analogues All cytotoxics have unwanted effects, par-
The vitamin D analogues calcipotriol and tacal- ticularly bone marrow suppression. This is
citol worl<well, and have rapidly found a place in rare with methotrexate, but may occur in an
routine management.Vitamin A analogues are idiosyncratic manner unrelated to dose. The
favoured by some authorities but are generally major problem with methotrexate is hepato-
less effective. There are few local side-effects toxicity, particularly fibl-osis with chronic use.
with either group (although vitamin D ana- Alcohol appears to exacerbate this tendency.
logues may burn on the face and in thcflexures), Younger patients require regular liver biopsies.
but calcium levels may be distul-bed if large Methowexate also inhibits spermatogenesis
quantities of vitamin D analogues are applied and is teratogenic. Its use is therefore irestl-icted
and patients using topical vitamin A may be t o severely affected patients.
advised t o avoid pregnancy because of
teratogenicity. Retinoids
Vitamin A derivatives help some patients with
Ultraviolet radiation psoriasis.The most commonly used is acitretin.
The use of UV light therapy is well established, Retinoids have a number of side-effects, includ-
the most effective wavelengths being in the ing dry lips, nose-bleeds, hair loss, hyperlipi-
medium (UVB) range. UVB must be used with daemia, liver function test abnormalities and
care because it also induces sunburn. Patients teratogenicity.
require doses that just induce erythema but do
not cause burning. The dose is then increased Systemic steroids
gradually Treatment is usually given twice In very severe psol.iasis, steroids may occasion-
weeldy until clearance is achieved. Adjunctive ally be necessary, butshould not be usedalone.
tar may mal<eUVB more effective.
UVB is theoretically carcinogenic (as is tar), Ciclosporin (cyclosporin)
but surprisingly few psoriasis sufferers develop This immunosuppressivedrugworkr extremely
skin cancers. well, even in very severe psoriasis. It is nephro-
toxic and very expensive.
Systemic therapies
Psoralen + ultraviolet A (PUVA)
'Psoralens' form chemical bonds with D N A in Treatment of clinical patterns
the presence of UV radiation.The most widely of psoriasis
used agent is 8-methoxypsoralen, which is
usually taken by mouth 2 h before exposure t o The choiceoftherapeutic regimen in psoriasis is
long-wavelength UV light (UVA), initially twice dictated by the type and extent of lesions, and
weel<ly. Protective glasses are worn to prevent by the effects on the patient's quality of life.
ocular damage.To reduce this risk, some units A balance will often have t o be struck between
now seal< patients' skin in a bath of psoralen the need for improvement and the incon-
solution.There is a significant long-term risk of venience andlor side-effects of the agent(s)
Ikeratoses and epithelia1 cancers with both concerned.
forms.
Chronic plaque psoriasis
Cytotoxic drugs Dithranol is a theoretical first choice, but the
The most effective and widely used cytotoxic patient's life-style, o r side-effects, may male it
is methotrexate, a folic acid antagonist. Most impractical. If so, vitamin D analogues o r topical
psoriasis responds t o a once weekly dose of steroids (with o r without tar and salicylic acid)
7.5-20mg. Other drugs include azathioprine are often used. UV radiation may help. If lesions
and hydroxycarbamide (hydroxyurea). become very extensive, o r if there are serious
Arthropathic psoriasis 75
Scalp psoriasis
Tar shampoos are helpful, but will seldom
control thick plaques alone. Tar gels may
help, butthe besttopical remedy is Unguentum
Cocois Co.-a mixture including tar and
salicylic acid. This is massaged in at night and
washed out the following morning. Topical
steroid lotions, with orwithoutsalicylic acid are
also used.
Nail psoriasis
Nail changes do not respond t o topical treat-
ment, and systemic drugs are seldom justified
for nails alone.
Guttate psoriasis
This is most easily treated with UV radiation
together with emollients and a tar-based
ointment.
Flexural psoriasis
Psoriasis in the flexures poses problems. Mild
taricorticosteroid mixtures may be effective,
but long-term use of topical steroids can cause choice is methotrexate, but ciclosporin (cy-
striae. Dithranol, used in very low concentra- closporin) also works well. When the condi-
tions, can be successful, but burning is common tion is stable, the dose should be gradually
and underclothes are stained. UVB and PUVA reduced and the drug stopped if possible.
generally fail t o ,reach the affected areasvitamin However, many patients relapse and require
D analogues help, but can sting. long-term treatment.
- Distalinterphalangealjointinwlvement
Seronegativerheumatoid-likejoint changes Reiter's syndrome
Largejointmono-orpolyarthmpathy
j Spondylitis This disorder. which frequently follows a diar-
rhoeal illness o r non-specific urethritis in HLA
627-positive individuals, is discussed in Chapter
above in descending order of frequency. Psori- 19. Occasionally sldn lesions lknown as'ikerato-
aticarthropathy is erosive and may result in joint derma biennorrhagicum' develop. Palmar and
destruction. plantar lesions may become very gross (Fig.
Psoriadcs who develop the spondylitic form 8.1 I), and lesions elsewhere are clinically very
are usually HLA 627 positive, and there is similartopsoriasis. Histologically.Ikeratoderma
some overlap between psoriatic arthropathy blennorrhagicum is indistinguishable from
and other seronegative arthritides. psoriasis.
Benign and Malignant
Skin ITClmouvs
Know ye not that a little leaven leoveneththe The first important principle is that, unless
wholelump?St Paul ( I Corinthians, 5 :6) the diagnosis is certain, some tissue should be
PI-eservedfor histology Failure t o do this will
mean missed malignancies, and is one explana-
Introduction and classification tion for patients who present with mysterious
of skin tumours lymphatic or distant deposits from unlmown
primary rites.
Lumps on or in the skin are extremely common
and the workload associated with them is rising Surgical removal o r biopsy
because: These techniques have already been described
I The age of the population as a whole is in- and illustrated (see Figs 2.1 & 2.2). Removal
cleasing (many sldn tumours are commoner in of small skin tumours is quick, simple and
rheelderly). economical. If the tumour is too large for pri-
2 Skin cancer is increasing in all agegroups. mary excision, take a small incirional biopsy,
3 There is increasing public awareness of the remembering t o cross the edge from normal
importance of skin tumours. t o abnormal tissue. There is no evidence that
Most skin tumours are benign, often repre- such a biopsy adversely affects the outcome,
senting only a cosmetic nuisance. However, it is although it is advisable t o avoid incisional
important t o distinguish these from malignant biopsy of invasive melanomas if possible (see
or potentially malignant tumours quickly and below).
effectively, as decisions about what should be
done about a lesion can only be made after a Curettage andlor
diagnosis t o this minimum level has been made. cautery ('C&C9)
The skin is a complex organ system, with This is a perfectly satisfactory method for the
both benign and malignant tumours described removal of superficial tumours.
for every component. Table 9.1 presents a
simplified version of the wide variety of sl<in
tumours.
Cryotherapy
The use of cryotherapy for tumours has
become very popular. It is ideal for superficial
skin tumours because it is quick and leaves rela-
tively little scarring. However, histological inter-
pretation of cryobiopsies is not easy and jt
shoul'd be used only if: the tumour is definitely
benign; o r an incisional biopsy has already The patient should be told t o expect
been performed. Cryotherapy is not appropri- blistering, followed by healing with crust for-
ate for melanomas. The best agent is liquid mation. The lesion should separate within 3
nitrogen. weeks.
Specific tumours 79
Epidermal cysts
There are three common forms of epidermal
cyst-pilar, epidermoid and milium.
I Common scalp cysts are correctly termed
'pilar' o r 'trichilemmal' cysts. There may be
several, and a familial predisposition is usual.
2 Epidermoid cysts may be found anywhere,
but are most common on the head, neck and
trunk.They often follow severe acne; there is a
cystic swelling within the skin, usually with an
overlying punctum.
Differential diagnosis. It is often confused with Differential diagnosis. Any soft-tissue tumour,
basal cell carcinomas o r other tumours. especially if there is no preceding history of
trauma.
Dysplasticlmalignant
epidermaltumours
Basal cell carcinoma (BCC)
The commonest malignant skin tumour is often
known as a'rodent ulcer'.
Morphoeic
A flat gomh pattern which results in a scar-
like appearance;It anbevery difficultto
know where the tumour begrns and ends,and
localinvasionw more common
superneid
Lesionsgrowformanyyearsandmaybemany
centimetres across;usualiysolitary;multiple
tumours may indicate previousarsenic
ingestion;characteristidlxa 'worm-like'
edgeisseen(Fig.9.10)
Figmentad
Pigmentationisusuallypatchybutmaybe
verydark anddense
I
Treatment. Excision, biopsy and radiotherapy or, Fig. 9.10 Superficialbasal cell carcinoma.
for superficial tumours, curettage, cryotherapy
o r photodynamic therapy; careful assessment
of morphoeic tumours is needed-a technique leading t o confusion with lentigo maligna (see
known as 'microscopically controlled surgery' below).
may be helpful; it is particularly important t o
deal adequately with lesions around the eyes, Treatment. Cryotherapy is best for small num-
nose and ears. bers of lesions; large areas on the face and scalp
can be treated with the topical antimitotic agent
Actinic or solar keratoses 5-fluorouracil; in the very elderly it may be best
These are areas of dysplastic squamous epithe- t o do nothing.
lium without invasion, but actinic keratoses do
have low-grade malignant potential and their Squamous cell carcinoma (SCC) in situ
presence indicates unstable epithelium. (or Bowen's disease)
Bowen's disease is a SCC confined t o the epi-
Clinicalfeatures. Red and scaly patches (Fig. 9.1 I) dermis, and is common in the elderly. Invasive
which characteristically wax and wane with change does occur but is rare.
time; many hundreds of lesions may occur in
heavily sun-exposed individuals. Clinical features. Usually a solitary patch of red
scaly skin, although multiple areas may occur;
Sites of predilection. Light-exposed skin, espe- Bowen's disease is asymptomatic.
cially the face, forearms, dorsa of hands, lower
legs and bald scalp. Variant. Erythroplasia of Queyrat- non-
invasive dysplastic changes may also occur on
Differential diagnosis. Some are pigmented, the penis, where the clinical appearance is of a
86 Chapter 9: Benign and Malignant SkinTumours
velvety red plaque. Although given a separate t o regional lymph nodes and beyond (especially
name, it is essentially the same as Bowen's lip, mouth and genital lesions). UV radiation is
disease elsewhere. important aetiologically, but other factors also
play a role: smoking in lip and mouth cancers;
Sites of predilection. Light-exposed skin; may wart virus in genital lesions.
occur on non-exposed areas such as the trunk.
Clinical features. These may be very varied, typi-
Differential diagnosis. There is a superficial re- cally either:
semblance t o psoriasis (Fig. 9.12), but the sur- I a keratotic lump,
face scale is adherent rather than flaky. Removal 2 a rapidlygrowing polypoid mass (Fig. 9.14), o r
of scale leaves a glistening red surface that does 3 a cutaneous ulcer.
not bleed. As arsenic was used in the past t o SCCs are often surrounded by actinic
treat psoriasis, keep an eye out for Bowen's keratoses.
disease in elderly psoriasis sufferers.
Similar changes on one nipple should always Sites of predilection. Sun-exposed sites; SCCs
suggest the possibility of Paget's disease (Fig. also develop on the lips (Fig. 9.15), in the mouth
9.13); a biopsy should be performed as there is and on the genitalia.
always an underlying carcinoma.
Differential diagnosis. Keratotic lesions may
Treatment. Should be treated by excision, curet- closely resemble hypertrophic actinic kera-
tage, cryotherapy o r photodynamic therapy; toses.
very large areas may require radiotherapy.
Treatment. Biopsy of any suspicious lesion;
Invasive squamous cell carcinoma definitive treatment is by surgical removal o r
SCCs are locally invasive, and may metastasize radiotherapy.
~ Lendgomalignamelanoma
The appearance of a nodule ofinvasive
- Clinicalfeatures:
rapidly growinglumps (Fig.9.18)
1 melanomawithinalentigomaligna. occasionallywarty (vemcous melanoma)
or non-pigmented (amelanotic melanoma)
Suprhdal s p r e a d i n g m e l a n m (SSM) * Sites ofpredilection:
i
~
I
ThecommonestintheUK; thetumourhasa
radialgrowthphasebeforemeinvasion
beejns
- may occur anywhere
Differential diagnosis.
other rapidly growingtumours
'
1
* Clinicalfeatures:
irregularlypigmentedbrownrDlackpatch
withanirregularedge(Fig.9.17)
mayitchorgive rise tomilddiscomfort
maybleed
Aaalmelanoma
Rare intheUK,butis muchmore commonin
other countries (e.g.Japan); it is virmally the
onlytypeofmelanoma seeninnsian orllfro- I
* Sitesofpredilection: Caribbean patients
most frequentlyon thelegin women and * Clinical features: I
t h e m n k i n men,butmayaccur anywhere apigmentedpatchon the sole or palm oran
* Differentialdiagnosis area of subungual pigmentation
I
naeviintheyoung * Differentialdiagnosis:
~
flat seborrhodc keratoses in olderpatients canbeconfused with aviralwan
mustbe distinguishedfmm haematoma
Nodular melanoma Some MMs ariseinpre-eldstingmelanacc
! Thhetumourexhibitsaninvasivegr~ naevialthough estimatesofthefrequencyof '
i pattern from the outset this vary from 5% to oyer50%.
~ ~~ ~~~~~~~~
permitted more sunny holidays abroad (and seems that the incidence of this varies from
at home), during which the most important countryto country.
'activity' is sunbathing. There are four recognized patterns of malig-
Periods of exposure t o very strong sunlight nant melanoma (see table above).
with sunburn are particularly risky and there
is evidence that childhood sun exposure may Treatment ofmalignant melanoma
be important. Some melanomas arise in pre- In understanding MM and its treatment it is
existing melanocytic naevi (see Chapter 10). It important t o realize that the prognosis is re-
* isanexistingmolegemnglargeroranew
one growing?Afterpubeqmoles usuallydo
- Is thelesionlargerthan 1 cmindiameter?
* Is thelesionlnfiamedaristhere areddish
not grow.(Thissignessentiallyrefersto
aduiw,rememberthatnaevimaygrowrapidly
inchildren (seeChapterl0))
--
edge?
Isthelesion bleeding.oozingorcmting?
Does thelesionitch orhurt?
* Doestheiesionhave animegularoutline?
Ordinaryrnolesarea srnooth.regular Any pigment& lesion,whethernemly arising
shape or alreadypresent,which exhibitsthreeor ~
Sites of predilection. Legs in the classical form; Diferential diagnosis. Lesions can be confused
anywhere in the aggressive form. with eczema o r psoriasis.
Diferential diagnosis. Other vascular lesions. Treatment. Biopsy is essential but can be difficult
t o interpret-DNA phenotyping of cells may
Treatment. Biopsy; symptomatic treatment with be of value; definitive treatment varies with the
radiotherapy. stage, but includes radiotherapy, PUVA and
chemotherapy.
Lymphomas
Lymphomatous involvement of the skin may be Extension from deeper tissues
secondary, for example in non-Hodgkin's B-cell and metastases
lymphoma. However, the skin may be the orig- Tumours of underlying structures, such as
inal site, especially in cutaneousTcell lymphoma breast, may invade the skin.The skin may also be
(often called'mycosis fungoides'). the site of metastatic deposits from internal
cancers such as bronchogenic carcinoma (see
Clinical features. Variable; some areas remain Chapter 19).
unchanged o r grow slowly for years; red, well-
circumscribed, scaly plaques and tumours
eventually develop (Fig. 9.20).
Introduction,94 Melanocytic naevi, 96
Epithelial and 'organaid'naevi, Vascularnaevi, 97
Ten thousondraw Iat oglonce (Wordsworth) portant: epithelia1 and organoid naevi, vascular
naevi and melanocvtic naevi.
Introduction
Epithelia1 and'organoid'naevi
Naevi are extremely common-virtually
everyone has some. However, the word'naevus' These are relatively uncommon developmental
can give rise to confusion. Much of the difficulty defects of epidermal structures: the epidermis
is due t o the term being used in several different itself; hair follicles; sebaceous glands.There are
ways, in addition t o that outlined below. Some two important types, the epidermal naevus and
writers use the word without qualification for the sebaceous naevus.
the commonest cutaneous hamartoma, the
melanocytic naevus (see below). The word is Epidermal naevus
also applied t o lesions that are not congenital at Circumscribed areas of epidermal thickening
all, such as the 'spider naevus' (which should may be present at birth or develop during
be a'rpider telangiectasis').This is complicated childhood; many are linear. Very rarely, there
further by some true'naevi' beingcalled'moles' are associated central nervous system (CNS)
o r 'birthmarks'. Thus, a lump described as a abnormalities.
'mole' may be a melanocytic naevus, but may Becker's noevus is an epidermal naevus that
also be any small skin lesion, especially if presents as a pigmented patch first seen at o r
pigmented,while'birthmark'is accurateenough around puberty onthe uppertrunkor shoulder,
as far as it goes, but many naevi develop after and which gradually enlarges and may become
birth. hairy.
We use the word 'naevus' t o mean a cuta-
neous hamartoma (a lesion in which normal Sebaceous naevus
tissue components are present in abnormal Sebaceous naevi are easily overlooked at birth.
quantities o r patterns). This encompasses They begin as flat, yellow areas on the head and
'naevi' which are not actually present at neck which, in the hairy scalp, may cause Iocal-
birth, because the cells from which they arise ized alopecia. Later, the naevus becomes thick-
are. ened and warty, and basal cell carcinomas may
Any component of the skin may produce arise within it.These naevi are best excised dur-
a naevur, and they may be classified accordingly ingadolescence.
(Table IO.I).We need only discuss the most im-
Epithelia1and 'organoid' naevi 95
* Epidermalnaevus
-
* Sebaceousnawus
Hairfolliclenaevus
- Spitznaevus
Bluenawus
vascuhr
Mehnorgtic Telangimatic
-
Congmiml
Congenitalmelanxyticnaevus
Mongolianbluespot
* Superli6dalcapillarynawus
* Deepcapillarynaevus
Rare telangiectaticdisorders
-
Acquired
JunctionaVcompoundd~al
naevus
Angiomotous
othal%SU~
Sunonshalonaevus Connectivetissue
* Dysplasticnaevus * Mastcell
*Fat
Melanocytic naevi
congenital
Congenital melanocytic naevus
One per cent of children have a melanocytic
naevus at birth.
These vary from a few millimetres to many
cenumetres in diameter. There is a rare, but
huge and grossly disfiguring variant, the'giant'
congenital melanocytic o r 'bathing trunli nae-
vus (Fig. 10. I). Fig. 10.2 Mangolianbluespot.
Congeniral melanocytic naevi are more
prone t o develop melanomas than acquired le-
sions. particularly the giant type. Prepubertal
Acquired
malignant melanoma is extremely rare, but
Acquired melanocytic naeuus
neal-ly always involves a congenital naevus.This
A melanocytic naevus is'acquired' if it develops
leads to a paradox: small, low-risl< naevi are
during postnatal life, a phenomenon that is so
easily removed but larger lesions with higher
common as t o be'normal'. Mostonly represent
malignant potential require extensive, even mu-
a minor nuisance, and'beauty spots' were once
tilating, surgery. Each case must be judged on its
highly fashionable.
own merits, and decisions must involve thefam-
Thetirstthingco understand is thateach nae-
ilyand thechild.
vus has its own life history.This will make the
terms applied t o the different stages in their
Mongolian blue spot
evolution clearer (Fig. 10.3).
Almostall children of Mongoloid extractionand
Thelesion (Fig. 10.4) is first noticed when lm-
many Indian and Afro-Caribbean babies are
mature melanocytes begin t o proliferate at the
born with a diffuse blue-blacl< patch on the
dermoepidermal junction (hence 'junctional').
lower back and buttocks (Fig. 10.2).There are
After a variable period of radial growth, some
melanocytes widely dispersed in the dermis
cells migrate vertically into the dermis ('com-
(the depth is responsible for the colour). The
pound'). Eventually all melanocytic cells are
area fades as the child grows, but may persist
within the dermis ('intradermal'). Different
indefinitely. Unwary doctors have mistaken
melanocytic naevi will be at different stages of
Mongolian blue spots for bruising, and accused
development in the same individual.
parents of baby-battering.
Most melanocytic naevi appear in the first
Vascular naevi 97
Fig.10.4 Acquiredmelanacyticnaevus.
Vascular naevi
sites are the nape of the neck ('salmon patches' Deep capillary naevi are less common but
o r 'stork marks'), the forehead and glabellar more cosmetically disfiguring than superficial
region ('stork marks' again) and the eyelids lesions. Most occur on the head and neck and
('angel's kisses'). Most facial lesions fade, but are usually unilateral, often appearing in the ter-
those on the neck persist, often hidden by hair. ritory of one o r more branches of the trigeminal
nerve (Fig. 10.8). They may be small o r very
Deep capillary naeuus extensive.
'Port-wine stains' o r 'port-wine marks' are A t birth the colour may vary from pale pink
formed by capillaries in the upper and deeper t o deep purple, but deep lesions show no
dermis. The deeper component gradually ex- tendency t o fade. Indeed they may darken
tends during life. with time, and often become progressively
Angiomatous naevi
In some accounts these lesions are classified
with capillary naevi, whereas in others they are
termed 'cavernous'. Most authorities acknowl-
edge that both capillary-derived elements and
larger, so-called 'cavernous' vascular spaces are
usually involved.
Strawberry naevus
Strawberry naevi arise very shortly after
birth. They may appear anywhere, but have a
predilection for the head and neck and the
napkin area (Fig. 10.9). Most are solitary, but
occasionally there are more. The lesion
grows rapidly t o produce a dome-shaped,
red-purple extrusion which may bleed if
traumatized. The majority reach a maximum
size within a few months.They may be large and
unsightly.
Spontaneous resolution is the norm, some-
times beginningwith central necrosis, which can
thickened. Lumpy, angiomatous nodules may look a1arming.A~a rule of thumb, 50% have re-
develop. solved by the age of 5 and 70% by age 7. Some
only regress partially, and a few require plastic
These lesions are most unattractive, and
patients often seek help. The newer types of surgical intervention.
The management, in all but a few, is expec-
lasers can produce reasonable results, and a
tant. It is useful t o show parents a series of
range of cosmetics can be used as camouflage.
pictures of previous patients in whom the lesion
There are three important complications.
has resolved
Specific indications for intervention:
I If breathing o r feeding is obstructed.
2 If the tumour occludes an eye-this will lead
t o blindness (amblyopia).
* An arsaciatedinmnanialvascular 3 If severe bleeding occurs.
malformation may result in fits,long-tract
signs and mental retardation.Thisisthe
4 If haemorrhage within a large tumour leads
SmrgeWeber syndrome t o consumption coagulopathy (Kasabach-
Congenitalglaucoma rnayormrinlaions ~
Merritt syndrome).
involvingtheareaof theophthalmicdivision 5 If the tumour remains large and unsightly
-
~
There is only one more beautiful thing than a Autosomal dominant ichthyosis
fine healthyskin,and that is a rareskin disease. (ichthyosis vulgaris)
(Sir ErasmusWilson. 180944) This is the commonest, and is often quite
A number of skin conditions are known to be in- mild. The scaling usually appears during early
herited. Many are rare, and will therefore only childhood.The skin on the trunk and extensor
be mentioned briefly.There have been maiorad- aspects of the limbs is dry and flaky, but the
vances in medical genetics in recent years, and limb flexures are often spared. Dominant
the genes responsible for many disorders have ichthyosir is frequently associated with an
been identified and their roles in disease atopic constitution.
clarified.
Several diseases in which genetic factors play X-linked ichthyosis
an important part, such as atopic eczema, pso- This type of ichthyosis only affects males. The
riasis, acne vulgaris and male-pattern balding. scales are larger and darler than those of dom-
are described elsewhere in the bool<. inant ichthyosis, and usually the trunl<and limbs
are extensively involved, including the flexures.
Corneal opacities may occur, but tliese do not
The ichthyoses interfere with vision. Affected individuals are
deficient in the enzyme steroid sulfatase-the
The term ichthyosis is derived from the Greek result of abnormalities in its coding gene.The
ichthys, meaningfish,astheskin has been likened majority of patients have complete or partial
to fish scales. The ichthyoses are disorders of deletions oithe steroid sulfatasegene.
keratinization in which cheskin is extremely dry Both X-linked ichthyosis and autosomal
and scaly (Fig. I I.I). In the majority of cases the dominant ichthyosis improve during the sum-
disease is inherited, but occasionally ichthyosis mer months.
may be an acquired phenomenon, for example
in association with a lymphoma.There are sev- lchthyosiformerythroderma
eral types of ichthyosis, which have different A bullous form of this condition is dominantly
modes of inheritance. inherited,andanon-bullour form recersively in-
102
Palmo-plantar keratoderma 103
herited. In both, the skin is scaly and erythema- helpful. The more severe types of ichthyosis
tous, and often has an offensive odour. often require oral retinoid therapy.
Treatment
Treatment consists of regular use of emollients Collodion baby
and bath oils. Urea-containing creams are also
This term is applied to babies born encased in a
transparent rigid membl-ane resembling colio-
dion (Fig. I I .2).The membrane cracla and peels
offafterafew days. Some affected babies havean
underlying ichthyotic disorder whereas in oth-
ers the underlying skin is normal. Collodion ba-
bies have increased transepidermal water loss.
and it is important that they are nursed in a
high humidicy environment and given additional
fluids.
Palmo-plantar keratoderrna
Ehlers-Danlos syndrome
Tuberous sclerosis
Peutz-Jeghers syndrome
Hereditary haemorrhagic
telangiectasia (Osler-Weber-
Rendu disease)
This inherited disorder of connective tissue, In this recessively inherited disorder there is
which has several modes o f inheritance, affects defective absorption of zinc. The condition is
elastic tissue in the dermis, blood vessels usually manifest in early infancy as exudative
and Bruch's membrane in the eye. The sl<in eczematous lesions around the orifices, and on
of the necl< and axillae has a lax 'plucked the hands and feet. Affected infantz also suffer
chiclen' appearance of tiny yellowish papules from diarrhoea. Acrodermatitis enteropathica
(Fig. 1 1.7). Retinal angioid streaks, caused by can be effectively treated with oral zinc
ruptures in Bruch's membrane, are visible on supplements.
fundoscopy. The abnormal elastic tissue in
blood vessels may lead t o gastrointestinal
haemorrhage. Angiokeratoma corporis
diffusum (Anderson-Fabry
disease)
Xeroderma pigmentosum
This condition is the result of an inborn error of
Ultraviolet (UV) damage t o epidermal D N A is glycosphingolipid metabolism. It is inherited in
normally repaired by an enzyme system. In an X-linked recessive manner. Deficiency ofthe
xeroderma pigmentosum, which is recessively enzyme alpha-galactosidaseA leads t o deposi-
inherited, this system is defective, and UV tion of ceramide trihexoside in a number of tis-
damage is not repaired.This leads t o the early sues, including the cardiovascular system, the
development of skin cancers. Basal cell carcin- kidneys, the eyes and peripheral nerves. The
omas, squamous cell carcinomas and malignant skin lesions are tiny vascula~.angiokeratomas
melanomas may all develop in childhood. In which are usually scattered over the lower
some cares there is also gradual neurological trunk, buttocks,genitaliaand thighs.Arrociated
deterioration caused by progressive neuronal features caused by tissue deposition of the lipid
loss. include thefollowing.
108 Chapter I I: Inherited Disorders
* Prematureischaemicheart disease
* Rendfailure Chromosomal abnormalities
Swerepain andparaesthesiaeinthehands
-
and feet
Cornealandlensopacities
Some syndromes caused by chromosomal ab-
normalities may haveassociated dermatological
omblems.
lncontinentia pigmenti
___\..^__.
An X-linked dominant disorder, incontinentia -
. ..
~
. - .. . .
pigmenti occurs predominantly in female in-
.
fants,as itis usually lethalin utero in males. Linear Down's syndrome:increased incidenceof
Bold was her face,and fair, and red ofhew, Normal pigmentary mechanisms have al-
(Chaucer, Thewife ofBath) ready been outlined in Chapter I. Humans have
a rather dull range of natural colours when
The complexlan ofthe skin and the colour ofthe
compared with peacocks o r parrots: normally
hair correspondto the colour ofthe moisture
only shades of brown and red. Brown is due t o
which the flesh attracts-white,or red,or black.
melanin, the intensity varyingfrom almostwhite
(Hippocrater)
(no melanin) t o virtually jet-black (lots). The
genetics of melanin pigmentation is autosomal
dominant.
Introduction:normal
Red is a bonus: only some people can pro-
pigmentary mechanisms
duce'phaeomelanin'. Red is much commoner in
some races (e.g. Celts) than in others (e.g.
Our sldn colour is important, and there are
Chinese).
many references t o it in prose and poetry. We
Most of human skin pigment is within
all note skin colour in our initial assessment
keratinocytes, having been manufactured in
of someone, and sldn colour has been used
melanocytes and transferred ln'melanoromes'.
to justify all manner of iniustices. Any depar-
There are racial differences in production, dis-
ture from the perceived norm can have
tribution and degradation of melanosomes, but
serious psychological effects and practical
not in the number of melanocytes (see Chapter
implications.
I).Thereare, however, importantgenetic differ-
A number of factors give rise to our skin
ences in the ability t o respond t o uh-aviolet ra-
colour.
diation, conventionally called'rkin types'.
-
* Haemoglobin
Exogenouspigmenoinorontheskin
dace
i
!
~
'l&el-alwayrbums.nevertans
7).pell-bumseasily.tanspoorly
* 7).peln-bumsoccasionally,tanseasily
7).pe N-neverbums.tans easily
* Endogenouslyproducedpigments(e.g.
* WeV-genetically brown (e.g.Indian) or
-
bilimbin)
Mdaninandphaeomelanb
Thelastm are themostimportantin
~ Mongoloid
i * 7)rpeVI-geneticallyblack(Congoidor
j Negroid)
dictatingourbasicskincolour . . --
1 10 Chapter 12: Pigmentary Disorders
-
* Phenylketonvria
mberoussdemsis
Hypochmmicnaevi
they are termed hypochromicnaevi.
Acquired
Acquil-ed hypopigmentation Is common and, in
--
kosvinea
Wtiligo
darker sldn, may have a particular stigma.This
-Suttonbhalonaevi
~kmloidleprosy
* Pityriasis(tinea)versicolor
is partly because the cosmetic appearance of
patchy hypopigmentation is much worse, but
also because white patches are inextricably
* Fityriasis alba linked in some cultures with leprosy. Histori-
LichensclerPwetatmphinu cally all white patches were probably classified
Drugs and chemicals: as leprosy: Naaman (who was cured of'leprosy'
occupationalleuccdema after bathing in the Jordan (2 Kings 5: 1-14))
self.inflictdiamgenic probably had vitiligo (see below).
Postinflammatolyhppigmenmtion
Vitiligo is the most important cause of patches
of pale sldn.The skin in vitiligo becomes depig-
Congenital mented and not hypopigmented. although dur-
Some individuals are born with generalized o r ing progression this is not always complete.
localized defects in pigmentation. Characteristically there is complete loss of
Albinism and phenylketonuria are due t o de- pigment from otherwise entirely normal skin
fects in melanin production. In albinos, the en- (Fig. 12. I). Patches may be small, but commonly
Hypopigmentation III
become quite large, often with irregular out- The organism causing pityriasis versicolor (see
lines. Depigmentation may spread t o involve Chapter 4 ) secretes azelaic acid.This results in
wide areas of the body. Although vitiligo can hypopigmentation, most noticeably after sun
occur anywhere, it is often strikingly symmetri- exposure.
cal, involving the hands, perioral and periocular Pityriasis alba (a low-grade eczema) is a very
skin. common cause of hypopigmentation in chil-
The pathophysiology is poorly understood. dren, especially in darker skins. Pale patches
In early patches melanocytes are still present, with a slightly scaly surface appear on the face
but produce no melanin. Later, melanocytes dis- and upper arms (Fig. 1 2.2). The condition usu-
appear completely, except deep around hair fol- ally responds (albeit slowly) t o moisturizers, but
licles.Vitiligo may be an autoimmune process: may require mild topical steroids.The tendency
there is an increase in organ-specific autoanti- appears t o clear at puberty.
bodies (as in alopecia areata, with which vitiligo Lichen sclerosus et atrophicus (see Chapter 15)
may coexist). usually affects the genitalia. O n other sites it is
Treatment is generally unsatisfactory.Topical sometimes called'white spot disease'.
steroids have their advocates, and PUVA can be Drugs and chemicals may cause loss of skin pig-
successful. Cosmetic camouflage may be help- ment.These may be encountered at work, but a
ful. Sun-screens should be used in the summer, more common source is skin lightening creams,
because vitiliginous areas will not tan. sold especially in areas with Afro-Caribbean and
In some, particularly children, areas may Asian populations. The active ingredient is gen-
repigment spontaneously. This is less common erally hydroquinone, which can be used thera-
in adults and in longstandingareas. Repigmenta- peutically (see below).
tion, when it does happen, often begins with Many inflammatory skin disorders may pro-
small dots coinciding with hair follicles.A similar duce secondary o r postinflammatory hypopig-
appearance occurs in Sutton's halo naevus (see mentation, due t o a disturbance in the integrity
Chapter 10). of the epidermis and its melanin production
Some of the stigma associated with hypopig- system: both eczema and psoriasis often leave
mentation is because tuberculoid leprosy is an- temporary hypopigmentation when they re-
other cause. The (usually solitary) patch of solve. However, inflammation can destroy
hypopigmented skin also exhibits diminished melanocytes altogether: in scars, after burns,
sensation. Pale patches may also be seen in the and in areas treated with cryotherapy (it is the
very earliest stages: so-called 'indeterminate' basis of 'freeze-branding').
leprosy.
1 12 Chapter 12: Pigmentary Disorders
Congenital
Hyperpigmentation i s prominent in neurofibro-
matosis: cafe-au-lait marks (Fig. 12.3) and axil-
lary frecklingare common. Speckled lenriginous
pigmentation is seen around the mouth and on
the hands in the Peutz-Jeghen syndrome, and
similar but more widespread lentigines may ac-
company a number of congenital defects in the
LEOPARD syndmme (Lentigines, Electrocardio-
graphic abnormalities, Ocular hyperteiorism.
Pulmonary stenosis. Abnormalitiesof thegeni-
talia. Retardation of growth and Deafness.).
Fig. 12.2 Pityliasis albaan thecheek. lncontinentiapigmenti is a rare congenital dis-
order which causes hyperpigmentation in a
whorled pattern, often with blisters and hyper-
Hyperpigmentation keratotic lesions, and sometimes other con-
genital abnormalities.
As with hypopigmentation, there are many
causes of increased skin pigmentation, including Acquired
excessive production of melanin, o r the deposi- Urticaria pigmentoso is most common in chil-
tion in the skin of several other pigments, such dren, but may affect adults. There is a wide-
as beta-carotene, bilirubin, drugs and metals. spread eruption of indistinct brown marks
The maior causes are as follows: which urticate if rubbed.The disorder is due t o
abnormal numbers of dermal mast cells.
Chloasma, o r melasma, i s commoner in
women than men. A characteristic pattern of
hyperpigmentation develops on the forehead.
cheeks and chin (Fig. 12.4). Provoking factors
include sunlight, pregnancy and the oral con-
traceptive pill, but chloasma may occur sponta-
neously. Treatment is difficult. Avoidance of
precipitating factors (especially sunlight and
oestrogens) may help. Topical hydroquinone
preparationsaresomedmes used.
Various drugs and chemicals can cause cuta-
neous hyperpigmentation (see Chapter 21).
In postinflammotory hyperpigmentation dis-
Hyperpigmentation 1 13
Ifo woman hove long hair, it is o glory to her. Changes in physical properties
(St Paul ( l Corinthians. I I : 15)) of scalp hair
Common physical changes which are seen in
The hairtakes root in the headot thesome time
hair are asfollows:
as the noilsgrow. (Hippocrates)
Introduction
. MonileW
%to*
Marieunnaaloped.
A large percentage of hairs suddenly stop
growing, enter the resting o r 'telogen' phase,
and fall out about 3 months later. Therefore,
* Dioordemofaminoaadmeebolism ask whether there has been any major upset
Scalpnaevi(especiallyepithelialor
in the appropriate period. Pull gently on hairs
organoid)
on the crown o r sides, and several will come
out easily. W i t h a hand lens the bulb looks
much smaller than normal. Telogen effluvium
settles spontaneously, but can unmask andro-
genetic alopecia (see below), and some patients
Very few of these conditions are amenable t o
find their hair never returns completely t o
treatment, but they require careful assessment,
normal.
often including microscopic examination of hair
Appropriate tests will exclude the important
S hafts.
systemic diseases listed below, and correct
treatment may restore hair growth.
-
Dfischairloarsarithno~-Ipskin
Telogen effluvium
* Thyroid disease
- %action
Mchotillamania
* Irondeficiency HairhwithabwrnulsaIpakJn
wlthout m n i n g
-
* Systemiclupuserythematom
Secondarysyphilis
Alopecia totalis
-
* Severepsoriasisorseborrhoeicdermatitis
nnea capitis (seeChapter4)
Umh mm'ng
Discoidlupus@ematosus
-
~mSm~Cic.lopcd.
* Liehenplanus
Circumsmii hairlossarithnonmldp Pseudopelade
* Alolopeciaareata .
* Cicatricialpemphigoid
Lupusvulgaris
1 16 Chapter 1 3: Disorders of the Hair and Nails
Many drugs can induce hair loss. Until recently there was no known treat-
ment, but there is some evidence that early use
of topical minoxidil may help both men and
women, and there are high hopes of a new
--Cytotoric agents
generation of selective antiandrogenic agents.
- Anti-thymid agents,especiallythiouradl
Anticoagulants
* VitaminAanalogues
Circumscribed hair loss with normal
scalp skin
Alopecia areata
Thhallium
The cause of this disorder is unknown but it is
All of these processes can be confused with probably an autoimmune process. Organ-
alopecia areata (see below) when the latter is specific autoantibodies (to thyroid, adrenal o r
widespread and rapidly progressive. gastric parietal cells) are often found in the pa-
Androgenetic alopecia (or common balding) tients' sera.
occurs in both men and women. It is due t o the
effects of androgens in genetically susceptible History. One o r more areas of baldness sud-
individuals. denly appear on the scalp, in the eyebrows,
In men, the process may begin at any age after beard o r elsewhere. It is most common in child-
puberty; however, it is much more common hood o r early adult life, although periodic recur-
from the thirties onwards, and by age 70 80% rences throughout life may occur.
show some hair loss. Hair is usually lost first
at the temples andlor on the crown, but there Examination. Patches are typically round o r oval
may be complete hair loss, sparing a rim at the (Fig. 13.1); the skin usually appears completely
back and sides.Terminal hairs become progres- normal, although there may be mild erythema; a
sively finer and smaller, until only a few vellus number of areas may develop next t o each
hairs remain.The extent and pace of this varies other, giving rise t o a moth-eaten appearance;
widely. close examination of the edge of a patch of
In women the process is slower and less alopecia areata reveals the pathognomonic fea-
severe, but causes much distress. Up t o half of ture -'exclamation mark hairs' -short hairs
all women have mild hair loss on the vertex which taper towards their bases (Fig. 13.2).
by age 50, and in some more severe thinning
occurs. There may be accompanying hirsutism Prognosis. Most patches regrow after a few
(see below). weeks, although further episodes can occur; ini-
tial hair growth may be white; occasionally, the In trichotillomania, hair is pulled, twisted o r
process spreads and may become permanent- rubbed out, and affected site(s) are covered in
if this state involves the whole scalp it is broken hairs of different lengths.There may be
termed alopecia totalis and if the whole body is psychologicalfactors (see Chapter 20).
affected, the name alopecia universalis is applied.
The nails may be affected in severe cases (see Hair loss with abnormal scalp skin
below). Psoriasis, seborrhoeic dermatitis and other in-
flammatory processes can cause temporary
Treatment. This is difficult, but intralesional hair loss: an important cause is tinea capitis (see
steroids may help, and topical sensitizers such as Chapter 4).
diphencyprone are also used. In some conditions fibrosis accompanies the
Chronic traction can also cause circumscribed inflammation, and this may result in permanent
alopecia, often around scalp margins (Fig. 1 3.3). damage t o hair follicles, and obvious loss of tis-
It is commonly seen in young girls with tight sue o r atrophy. This is known as scarringo r cica-
'pony tails', Sikh boys and Afro-Caribbean tricial alopecia.
children whose hair is dressed in multiple little Examination of the rest of the skin may pro-
'pigtails'. vide important clues.
ll8 Chapter 13: Disorders ofthe Hair and Nails
-
* Prominent pluggingofthehair follicles
Look for lesionson the face
Especially ininternational residents
-
Cicatricialpemphigoid
AloDecia follows blisterinQ
distinguishing feacures
-
andmgeneticalopeciaabwe)
Minorend&edisnubances.espedany
-
Nail changes may be non-specific, o r character-
polycysticova~~syndmme istic of specific processes. They may occur in
Drugswith androgenicactivitg
isolation, but the nails are abnormal in several
Virilidngtumours
disorders.
Nail abnormalities 1 19
Congenital Acquired
* Especiallydisordersofkeratinization,e.g.
Darier'sdisease -
* Psoriasis
EczemaJdematitis
- Ectodermaldysplasias
Due toscamng,e.g. dystrophic
epidennolysis bullosa
Lichenplanus
* Alopecia areatdmtalis
* Fungalinfections
~~
-
Brittleness
lncreaseswithage
0 Seeniniron deficiency(seealsokoi1onychia)
--
* whitenails-associatedwithcirrhosis
Pale-anaemia
Halfredhalfpale-renal disease
and thyroid disease Sulfuryellow-fungalinfection
* UniformyeIlow-'yellow nail syndrome'
-
!
m g k s (Q=b-nychia) (+bronchiectasis andlymphoedema)
-
* Commonandoftennon-spedfic
May result fiomwidespreadpitting(see
below)
Green-blue-Pseudornonas infection
* Brown-black-melanoma, haematoma
linearbrown-naevus
-
Bean's h e a
Horizontal~~oovesduemmajorillners Nailswithaconcaveuppersurface
Fits
* Classicalfeatureofpsoriasis
.
(spoon-shaped)
causes:
iron deficiency
* severe alopeciaareata (smaller,morewenly inherited
distributedtianin psoriasis)
* Eczemddennatitis (coarsedentsand Wpshbaardnui
irregular pits) * Habitualpickingofnail foldleads to surface
ridg5ng
Ony&olpis(Fig. 13.4)
Liftingofnailplateoffnailbed Onphogryphads
* Causes: * Grossly thickened,distorted nails (Fig. 13.5)
psoriasis often due toneglect
fungalinfection (see Chapterg
thyrotoxicosis wqgium*
space-occupyinglesion(e.g. exostosis or * Damageleadstoepitheliumencroachingon
- tumour)
Maybenootheridentifiableabnormality
present
nail surface
* Cause-lichen planus
Lass of"*
-
Clubbing
Signofpulmonary,liverorthyroiddisease:
may be familial
* Causes:
ptevgium
scamng, e.g. Stevens-Johnson syndrome
severe inflammation,e.g. pustular
UiSCU1-h psoriasis
Whitemarks-commonnonnalwiant
120 Chapter 13: Disorders of the Hair and Nails
-
shy.ial
Cold.heac.friction.oedema
Skindisorden
-
Cmgmial
Epidermolgsisbulloaa
fnfcctioar(seeChap3and41
~ccelid Md
* Impetigo Bullae are a major f e a m in:
* Pemphigus
.
yiml
chickenpm
Herper wster
* Bullouspmphigoid
* Cicauicialpemphigoid
-- Herpessimplex
Smallpoxandvaccinia
Hand,footandmouthdiKase
-1
* l5neapediswithpompholyx
-(-Chapm$
* Insectbites
-
frusemide,nalididcacid (light-induced)
Drug-inducedpmphigusandpemphi~id
Fixeddrugeruptions
Causes 123
injury and are common causes of blisters, as is 2 Loss of adhesion of epidermal ceHs
extreme friction (e.g. the feet ofvigorous squash ('acantholysis').
players o r joggers). Severe, acute oedema ofthe These changes may be just above the basal
lower legs may also produce tense bullae. layer (pemphigus vulgaris; Fig. 14.1) o r higher in
the epidermis (pemphigus foliaceus; Fig. 14.2).
Arthropods The commonest variant is pemphigus vulgaris,
Remember that insect bites very commonly which presents with flaccid blisters and ero-
present as tense bullae (see Chapter 5). In the sions (Fig. 14.3).These may be anywhere, but in
UK, this is most common in late summer and over 50% of patients the disorder involves the
early autumn (fall). mouth (Fig. 14.4). Perineal lesions are also com-
mon.The blisters rupture easily and the result-
Drugs ing erosions heal very slowly, if at all. A highly
Several drugs cause blistering (see above). Blis- characteristic feature is the Nikolsky sign: skin at
ters caused by nalidixic acid occur on the lower the edge of a blister slides off when pushed by a
legs following sun exposure. Fixed drug erup-
tions may blister (see Chapter 2 1 ).
Skin disorders
Primary skin disorders giving rise t o bullae may
be congenital o r acquired. In some, bullae are an
important o r integral part of the clinical presen-
tation. In others, blisters may occur but are not
the most prominent o r constant feature, and
the reader should consult the appropriate
chapter for further information.
Congenital
Epidermolysis bullosa
Although very rare, this is an important group of
Fig.14.1 Pemphigus vulgaris: split just above the
disorders. Babies are born with fragile skin that basal layer,with overlyingacantholysis of
blisters on contact. There are several variants, epidermal cells.
with splits at different levels in the skin; all are
unpleasant and some are fatal.
Diagnosis requires electron microscopy t o
determine the level of the blister.
The differential diagnosis of blistering in a
neonate must also include a number of other
disorders:
I Impetigo (pemphigus neonatorum).
2 Staphylococcal scalded skin syndrome (see
below).
3 lncontinentia pigmenti (see Chapter I I).
Acquired
Pemphigus
The cardinal processes in all forms of pemphigus
are: Fig.14.2 Pemphigus foliaceus: similar changes to
I A split within the epidermis. those in Fig. 14.1but higher in the epidermis.
1 24 Chapter 14: Bullous Disorders
Treatment. Treatment must be vigorous. Before Bullous pemphigoid and cicatricial pemphigoid
systemic corticosteroids were available most Bullous pemphigoid is much commoner than
patients died, often after a long and debilitating pemphigus. More than 80% of patients are aged
illness. over 60.
High doses of prednisolone (60- 120 mg Bullae are the key feature, but are not always
daily) are used. The dose is gradually reduced present initially: the process may begin with a
when new blistering has ceased (usually in about non-specific phase known as 'prepemphigoid',
4-6 weeks). lmmunosuppressiveagents such as characterized by intense irritation and well-
azathioprine, chlorambucil, cyclophosphamide defined, slightly elevated, erythematous areas.
o r methotrexate may be added as steroid- The bullae, which are usually numerous, are
sparing agents. tense and dome shaped, and may be blood filled
Good nursing and metabolic management (Fig. 14.6).They vary from a few millimetres t o
are crucial because pemphigus patients may be several centimetres in diameter and often arise
systemically ill. Widespread erosions cause loss on urticated erythema as described above, but
of protein and fluid, and secondary infection is are also seen on normal skin. Although the le-
common. If the mouth is severely involved, pa- sions may appear anywhere, there is a marked
tients cannot eat and may be severely catabolic. predilection for the limbs. Oral involvement
l26 Chapter 14: Bullous Disorders
occurs in about 30%.When blisters burst, heal- Bullous pemphigoid usually responds rapidly,
ing is usually rapid. Some blisters do not burst, and maintenance therapy with small doses is
and the fluid is simply reabsorbed. Scarring is usually possible. The condition appears t o be
rare, but there is a distinctive variant, charac- self-limiting in some. Cicatricial pemphigoid is
terized by marked scarring, known as cicatricial much less responsive.
pemphigoid. This condition has a predilection
for oral, conjunctival and genital epithelium. Dermatitis herpetiformis and linear IgA disease
The diagnosis in both forms of pemphigoid Dermatitis herpetiformis is uncommon. Its
requires: importance lies in its ability t o cause severe itch-
I A biopsy for histopathology. ing, and i t s association with gluten-sensitive
2 A biopsy for immunofluorescence. enteropathy.
3 Serum for indirect immunofluorescence Clinically, the cardinal features are pruritus
(less valuable). and grouped erythematous papules and ves-
The pathologicalfindings are as follows: icles, found most typically on the elbows
(Fig. 14.9) and extensor surfaces of the fore-
~ * Asubepidemal blister(Fig.14.7)
* Alinearbandoflgc andC3 at thebasement
membrane zone (Fig.14.8)
* Acirculatinglgcantibodytobasement
membranein 70%ofpatien+swith bullous
pemphigoid
1 NOcircu~atingantiiyinci&txicial
pemphigoid
arms, knees and shins, buttocks, shoulders Dermatitis herpetiformis should be con-
and scalp. sidered in any patient with atypical eczema
The intense itching may result in excoria- o r pruritus localized t o the areas mentioned
tions, secondary eczematization and lichenifica- above.
tion and it can be difficult t o find intact vesicles The diagnosis requires:
o r bullae. I A biopsy of a blister or, preferably, a new pink
papule for histopathology.
2 A biopsy of normal skin for immuno-
fluorescence.
3 A jejunal biopsy.
The main pathological findings are as follows:
* Arubepidermalblisterwhichis
indistinguishable,whenfully formed and
intact,from that seen in bullouspemphipid
Howeuer.invelyearly,prevesicularlesions
1
(hencethepinkpapule).orattheedgeofa
vesicle,there are small neutrophil
microabscessesin dermal papillary tips (Fck
14.10).Theseare pathognomonic
GranularlgAin the dermalpapillarytipson
immunofluorescence(Fig.14.11)
- Therearenocirculatingantikdies
Gutchangesrangefroman increasein
lymphocytenumberstovarious gradesof
I
villous atrophy
Fig.14.10 Dermatitis
herpetiformis: papillary tip
microabscesses as well as a
subepidermal blister.
128 Chapter 14: Bullous Disorders
preservatives may be important, but in our I Dermographism: weals appear after scratch-
experience (and that of most dermatologists) marks (Fig. 15.2); this may occur alone o r with
this is only true of a minority of patients. other forms of urticaria.
2 Pressure (delayed): weals develop up t o 24 h
The physical urticarias after pressure is applied.
Several physical insults may trigger urticarial 3 Cholinergic urticaria: affects young men;
responses: sweating is accompanied by small white weals
with a red halo on the upper trunk.
4 Cold.
5 Water.
6 Sunlight.
7 Heat.
Hereditary angioedema
In this very rare autosomal dominant
condition:
I C I esterase inhibitor is lackingo r defective.
2 There are sudden attacks of angioedema
which can be life-threatening.
3 The gut may be affected, giving rise t o spasms
of abdominal pain.
Urticaria pigmentosa
Abnormal accumulations of mast cells result in
multiple pigmented macules that urticate on
being rubbed (see also Chapter 12).
Treatment of urticaria
Erythema multiforme
If a possible trigger
.. can be elicited from the his-
tory, it should be avoided. Aspirin should be
Theclassiclesion oferythemamultiforme isthe
banned in anyone prone t o urticaria.
'iris' or'target' lesion (Fig. 15.3): a round or oval
Mosttypes of urticariarespond t o H, antihis-
area of erythema, with a dusky, purplish centre.
tamines although some of the rarer, physical
Sometimes the centre becomes paler and a
forms do n0t.A large rangeofagents is available.
blisterformr.
many of which cause CNS depression, but
several newer antihistamines have litde o r no
History. Lesions appear suddenly, enlarge over
sedative effect (e.g. loratidine, cetirizine, fexo-
the course of afew days, and fade (often leaving
fenadine).Theseare now drugs offirst choice. It
pigmentary disturbances). The whole process
may help t o add an H, antagonist (cimetidine,
settles in about 3 week. Repeated episodes are
ranitidine).
rare, but can be triggered by herpes simplex
It is sometimes necessary to use other
(see below)
agents, such as systemic steroids and adrenaline
(epinephrine) (see below)
Aetiology Erythema multiforme may occur out
of the blue, but there are several recognized
triggers.
Acute attacks-afewdays'treatmentis
-
usually sufficient
ChroNcurticana-@eado~of
antihistamine which suppresses the eruption * Herpessimplex-the commonesttrigger.as
campletely,maintainthisdosefo~several herpes maybe recurrent,so may herpes-
months;graduallywithdrawtreatment related erythema multifonne
Angiwdema-may requireparenteral * Othervirures-orf. hepatitis,mumps
therapywith adrenaline (epinephrine).
-
an~israminesand steroids
Anaphylaxis-adrenaline (epinephrine)is
- Radiotherapy
Cancers
* Connectivetissuediseases
required * Awidevarietyofdrugs
fHereditavangioedema-does not respond
to antihistaminesor steroids; danazol works
by increasinglevelsofthe missingenzyme;
purified enzyme preparationsare available Examinotion. The distribution characteristically
foracuteattacks
includes extensor surfaces of arms and legs, but
most important diagnostically is involvement of and cause ocular scarring. Patients occasionally
palms and soles. die of severe bronchopulmonary involvement
o r renal failure.
Pathology.Theprocesrisavarculitisand themore
serious the vascular damage, themore dramatic Treatment. Close attention must be given t o
are thechanges.When really severe, theepider- fluid balance and nutrition. The role of sys-
mis becomes necroticand bullae may form. temic steroids is controversial, because the
morbidity from steroids may outweigh that of
T-eotment. Erythema multiforme is self-limiting. the disease.
and treatment is not usually required.
Stevens-Johnson syndrome
A t is mostextremeerythema mulriformecaus-
es a major systemic disturbance. There is an
acute onset, with severe inflammation of con- These terms (either will do) are used to de-
junctivae, mouth and genitalia (Fig. 15.4). which scribes state in which most of theskin becomes
may prevent normal eating, affect micturition red, inflamed and scaly (Fig. 15.5).
Lichenscierosusetatrophicus (oftenshortened
to lichen sclelnsus o r LS et A) is a disorder of
unknown aetiology.
Clinical features:
I White, atrophic patches on the vulva.
perineum and perianal skin, or glans penis and
foreskin.
Aetiology. Lichen planus i s aT-cell-mediated at- 2 Similar plaques may develop elsewhere.
tack on the epidermis, similar changes being 3 Purpura and blistering may appear.
seen in graft-versus-host reactions. However. 4 Vulval lichen sclerosus easily becomes
the cause of lichen planus in most instances eroded and haemorrhagic, with severe sore-
remains a mystery. ness and irritation.
Treatment. Potent topical steroids usually sup- Complications. Vulvo-vaginal stenosis; develop-
press irritation; very extensive o r severe oral ment of rquamous cell carcinoma.
disease may need systemic steroids o r
ciclosporin (cyclorporin). Childhooddisease. Lichen sclerosus in prepuber-
tal girls often presents with dysuria and pain on
defaecation. It may be mirdiagnosed as sexual
Lichen nitidus abuse, but lesions are usually easy t o diagnose
(Fig. 15.8), and parents and child can be reas-
Probably avariant of lichen planus, this uncom- sured. The prognosis of childhood disease is
mon disorder produces clusters of tiny, asymp- good, as many clear at puberty.
tomatic papules.
Disease in moles. Lichen sclerosus may be seen
on theglans and prepuce (sometimes called'bal-
136 Chapter 15: Miscellaneous Disorders
..
- Sunburn
~~lymolphiclightemption
* Photosensitiveeczema
* Porphyrias
* Solarurticaria(seeabave) * Pellagra
!
* ACtiniCpNrigO
* JuvenileSpringeNpion
Hvdroavaccinifome
- Xerodennapigmentosum
Phytophotdematitis
Light-induced skin disease 139
Fig. 15.12 A
phytophotodermatitis.
141
142 Chapter 1 6: Vascular Disorders
Tveatment
Varicose eczema may be treated with mild po-
tency topical steroids. Duplex sonography is a
valuable investigation, and assessment by a vas-
cular surgeon is an important aspect o f manage-
ment, as some patients benefit significantly from
surgery on incompetent superficial veins. In ad-
dition, it is essential t o assess the arterial supply
in patients with leg ulcers because they may have
a remediable arterial abnormality. It is n o t un-
common t o discover both venous and arterial
pathology in individuals with leg ulcers.
Another important aspect of the manage-
ment o f venous ulcers is reduction of venous
hypertension and oedema by compression
bandaging. It is vital, however, t o establish that
Fig.16.1 Lipodermatosclerosis. the arterial supply t o a limb is adequate (by
Doppler studies) before using compression ders, including rheumatoid arthritis and sys-
bandaging. temic lupus erythematosus (SLE), may produce
Secondary infection, often with a mixed bac- legulcers.
terial flora, occurs in the majoricy of venous
ulcers. However, systemic antibiotic therapy Neoplastic ulcers
is not necessary unless there is associated Basal cell carcinomas and squamous cell carci-
cellulitis (see Chapter 3). nomas arisingon the legs may resemble, and be
There are numerous agents which have been mistaken for, venous ulcers. However, they usu-
marketed as topical therapies for leg ulcers. ally occur above the ankle region. If there is any
including alginate, hydrogel and hydrocolloid suspicion that an ulcer is neoplastic, a biopsy
dressings, but a simple regimen of regular should be performed.
cleansing with saline, followed by the applica-
tion of a topical antibacterial dressing such Haematological disorders and
as chlorhexidine gauze, is often adequate if leg ulcers
combined with compression bandaging. Uncommon causes of leg ulcers include heredi-
When a venous ulcer has healed i t is impor- tary spherocytosis, sickle cell anaemia and
tanttomaintain compression by wearingagrad- thalassaemia. The mechanism of ulceration in
uated compression stocking. these conditions is related t o tissue hypoxia
due to blockageof sl<incapillaries by abnormally
lschaemic ulcers shaped red cells.
lschaemic ulceration is usually a manifestation
of atherosclerotic peripheral vascular disease.
Typically, ischaemic ulcers occur on the dorsum Vasculitis
o r the sides of the foot, between the toes, o r
on the heel. Pedal pulses are reduced o r absent. Classification of vasculitis is difficult, but a sys-
and Dopplel- studies will demonstrate im- tem based on the size of vessel involved and the
paired blood flow. lschaemic ulcers are usually role played by neutmphils, lymphocytes and
painful. g~ranulomatousprocesses is relatively straight-
The advice of a vascular rulgeon should be forward.Triggers of vasculitis include immune
sought. complexes, bacterial and viral disease, and
drugs. Heat and cold damage are also respons-
Vasculitic ulcers ible for vascular changes. Clinically, vasculitis
Vasculitis associated with a number of disor- may present as urticaria, livedo reticularis,
144 Chapter 1 6: Vascular Disorders
-
Polymorphonudear
'Auer& vasculitis (leukocytadastic
vasculms)
o r bullae.
Histologically, there is fibrinoid necrosis of
small blood vessels, and a perivascular infiltrate
* Behcet'sdisease composed predominantly of neutrophil poly-
morphs. The perivascular tissues also contain
-
Lymphaytic
Dmgemp&ans
extravasated red cells, and fragments of poly-
-
* Erythemanodosum
filblains
morph nuclei (nuclear dust).These changes are
initiated by deposition of immune complexes in
small vessels, complement activation, and pro-
--
Granulmatous
Wepener's granulomatosis
Nodularvasculitis
duction of polymorph chemotactic factors.
Polymorphs attracted t o the area release en-
zymes which damage the vessel wall. Drugs,
bacterial o r viral infections may act as the anti-
Large-1 genic triggering factor, but often the initiating
mlymorphonuclar
* Polyarteritisnodosa factor is not discovered.
The joints, kidneys and gastrointestinal tract
Lymphocyfic may be affected, and it is important t o check the
* Lupuse@ematosur urine for microscopic haematuria. Henoch-
Schonlein purpura (anaphylactoid purpura)
Gmnulommous is the name which has been given t o a systemic
* Giant-cellarteritis
allergic vasculitis occurring predominantly in
Behqet's disease
The principal features of this disorder are recur-
rent, severe oral and genital ulceration, and
uveitis. Skin lesions include erythema nodosum
and pustules at sites of minor trauma such as
venepuncture sites.
dermatologists call this disorder 'equestrian fat, occur on the legs. These may ulcerate.
cold panniculitis'. Other organs are not involved.
Treatment for chilblains is not very satisfac-
tory. The best management is prophylaxis, by Large vessel
wearing warm gloves and thicksocks, and, in the Polyarteritis nodosa
case of the equestrian, thermal underwear and Polyarteritis nodosa (also known as periarteri-
thick jodhpurs. tis nodosa) is an uncommon type of necrotizing
vasculitis which affects small and medium-sized
Wegener's granulomatosis arteries throughout the body. Immune com-
This is a rare form of necrotizinggranuIomatous plexes appear t o cause this disorder, and the
vasculitis affecting principally the small arteries triggering antigens may be infections o r drugs.
of the respiratory tract, and associated with Manifestations include pyrexia, weight loss,
glomerulonephritis. Skin lesions take the form arthralgia and myalgia. The most significant
of a nodular vasculitis, sometimes with ulcera- clinical sign is the presence of cutaneous o r
tion. I t is associated with the presence of anti- subcutaneous nodules along the course of
neutrophil cytoplasmic antibodies (ANCA). superficial arteries. Vessel damage results in
aneurysm formation. Livedo reticularis and
Nodular vasculitis skin ulceration are other features. There may
This is an ill-defined condition, predominantly be renal, gut, cardiac and nervous system
affecting middle-aged women, in whom inflam- involvement.
matory nodules, produced by a granulomatous There is a type of polyarteritis nodosa which
vasculitis in the deep dermis and subcutaneous affects the skin alone. Livedo reticularis and
Vasculitis 147
cutaneous nodules occur on the legs, usually Lupus erythematosus (see Chapter 17)
below the knees.
Temporal arteritis (giant cell arteritis)
Treatment. Polyarteritis nodosa is treated with Skin changes are rare, but ulceration may occur
systemic steroids and immunosuppressive on the temporal and parietal regions of the
drugs. Purely cutaneous polyarteritis usually scalp.
responds t o small doses of systemic steroids.
Connective Tissue Disorders
Lupus erythematosus
Investigations Skin
The diagnosis can be confirmed by skin biopsy The skin changes are as follows:
Histology shows a periadnexal lymphocytic
infiltrate. liquefaction degeneration of the basal
layer of the epidermis, follicular plugging, and
hyperkeratosir. Direct immunofluorescence
* Wolaceouserythema afthe face andv-area
of lesional skin reveals the same pattern of aftheneck(Fig.l7,3).Thisissaidtoresemble
immunoglobulin deposition seen in SLE (see the colour of the heliotrope flower,and is
above). referred to as 'heliotrope erythema'
Deattnent
Potent fluorinated topical steroids are helpful
-
* Periorbitaloedema
Erythemaon thedorsaofthehands,and
linear erythema on thedorsa ofthe fingers
(Fig. 17.4). Erythematous papules (Gcmon's
in many cases, but ifthey are ineffective, intrale-
papules) over the knuckles
sional injection of triamcinolone, o r oral the- * Prominent,raggedcuticlesand dilated
rapy with the antimalarial chloroquine may be capillariesin the proximal nail folds (Fig. 17.5)
required. Light-exposed areas should be * EIythemaoverknees and elbows
protected by a sun-screen with a high SPF. * In childhood.cutaneousMsculitisleads to
Where there is extensive involvement of facial ulcerationof the skin.particularlyinthe
skin, the use of cosmetic camouflage can be of
axillae and groins
benefit.
Dermatomyositis
Heliotrope
A flower resemblingthepole violet,
Which, with the Sun,though rooted-fost,doth
move
And,beingchooged,yet changeth not her love
(Ovid)
Dermatomyositis is an autoimmune inflamma-
tory diseaseof sldnand muscle which may occur
in childhood or in adult life. There are differ-
ences in the manifestations of the disease in
these two agegroups.Vasculitisand the late de-
velopment of calcinosis are features ofthe child-
hood disease which are n o t seen in adults. In
some adults dermatomyositis is associated
with systemic malignancy, whereas there is no Fig. 17.3 Faciale~ytl~emaindermatomyositis
association with malignancy in the childhood
disease.
Dermatomyositis 15 1
'ITeatment Morphoea
In dermatomyositis associatedwith malignancy, This isadisorderofunknownaetiologyin which
there is usually marked improvement when the there is sclerosis of the skin. I t may be sub-
neoplasm is excised.A relapse of the dermato- divided clinically into the following types:
myositis signals a recurrence. I Circumscribed.
The mainstay of therapy is oral corti- 2 Linear.
costeroids. If the response t o steroids is poor, 3 Frontoparietal (en coup de sabre).
immunosuppressives such as azathioprine, 4 Generalized.
methotrexate o r cyclophosphamide may be of
benefit. Where there is severe muscle involve- Circumscribed
ment, physiotherapy is an important adjunct t o This is the commonest clinical presentation
drugtherapy, in o~.dertominimizecontractures. of morphoea. Solitary 01-multiple indurated
plaques develop, predominantly on the trunlc
Initially, affected areas of skin have a violaceous
hue, but gradually become thickened and ivory
in colour (Fig. 17.6).The surface is smooth and
Scleroderma means'thiclening of the sl<in', and shiny. Eventually,usually after many months, the
is a term applied t o agroup of diseases in which sclerosis I-esolves, leaving atrophic, hyperpig-
there is sclerosis of the skin and destruction of mented areas.
hair folliclesand sweatglands. Scleroderma may
be an isolated cutaneous phenomenon, when it Linear
is called 'morphoea', o r a cutaneous compo- Linear morphoea usually affecrs one limb, often
nent ofa multisystem disorder. extending i t s full length. In childhood, it can sig-
[ c;A;.-.>zLrw. r-r.x.&
, , I , . . , j
, Z::+*'Q;';'&:"&&
-
systemic involvement
Systemicsclerosis mtaneoussclemsisin
associationwith a vasculopathy of small
oftheskinas amanifestalionofthe toxic
effectsof certain chemicals
* Pseudoscleroderma:sclerasiooftheskin
arteries produangmultiorgansystemic associateZwithanumberofdiseasesother
disease than morphaea orsy~temicsclemsis
1- ~~ ~ ~- ~ ~~
Scleroderma 153
nificantly impair the growth of the limb, and pro- Systemic involvement
duce severe flexion deformities of large joints Gastrointestinal. In the oesophagus, damage t o
and digits. the myenteric plexus leads t o hypomotility of
smooth muscle and later t o atrophy and
Frontoparietal (en coup de sabre) fibrosis, resulting in impaired peristalsis. The
Resemblingasabrecut acrossthe scalp and fore- gastro-oesophageal sphincter mechanism is
head, this type of morphoea is a considerable also impaired, leading t o gastro-oesophageal
cosmetic problem. A linear, depressed, sclerotic reflux, oesophagitis, and eventual stricture
area extends from the face into the scalp, and is formation. Symptoms of oesophageal reflux
associated with loss of hair along its length. are common. Dysphagia usually indicates the
development of oesophageal stricture.
Generalized Atrophy and fibrosis of the smooth muscle of
There is extensive sclerosis of the skin on the the small bowel leads t o impaired peristalsis,
trunk and limbs. Flexion contractures restrict and the resultant relative stagnation of small
limb movement, and if the chest is severely bowel contents predisposes t o bacterial over-
affected breathing may be impaired. growth as colonic bacteria move upstream into
the small intestine. Gut bacteria deconjugate
Deatrnent bile salts (which are essential for micelle forma-
There is no effective treatment for morphoea. tion) and this leads t o fat malabsorption and
In linear morphoea on the limbs, physiotherapy steatorrhoea. Occasionally, patients pre-
is essential t o maintain joint motility, and sent with a picture simulating acute intestinal
othopaedic surgery may be necessary. Plastic obstruction.
surgery can be of benefit in frontoparietal Similar pathology affects the large bowel
morphoea. and leads t o the formation of multiple wide-
The natural history of morphoea is gradual mouthed pseudodiverticula.
spontaneous resolution.
Pulmonary. An inflammatory alveolitis is fol-
Systemic sclerosis lowed by pulmonary fibrosis, and disease of
This is a condition of unknown aetiology in small pulmonary arteries leads t o pulmonary
which sclerotic changes in the skin occur as one hypertension and cor pulmonale.
component of a multisystem disorder associat-
ed with a vasculopathy of small arteries. The Renal. Fibrinoid changes in arteries and arteri-
skin changes affect predominantly the face and oles are associated with proteinuria and hyper-
hands. tension. Renal involvement is usually mild, but in
Face HandS
The fadal skin is sclerotic andbound to
underlyingsrmmues.pmducingatight, -
* Raynaudkphenomenon
Tightsclemticsldnp~ucing~~~e
shinyappearance,arithlossoffadalwrinkles.
abeaked nose,andresoictionofmouth
opening(Fig.17.7)
-
mntramres ofthe digits (sclercdactyly)
FingerpulpinfarcrsprDdudngsmaIl,
painfululcerr (Fig. 17.8).Theseinfarctiw
* Perioralfunwiing(pme-stringrnouth) changeslead topropssive pulpatrophyand
Facial telangiectasia resorptionofthe underlyingterminal
* Lossoflipvermilion phalanges
Calcinosk(Fig.17.9)
~~~ . .... .
154 Chapter 1 7: ConnectiveTissue Disorders
some cases it is severe and rapidly progressive, Cardiac. Myocardial fibrosis, conduction dis-
and leads t o renal failure. orders and a variety of electrocardiographic
(ECG) abnormalities have been described.
Nervous system. Neurological involvement is
uncommon, but carpal tunnel syndrome and Hepatic. There is a significant association
trigeminal neuropathy have been reported. between systemic sclerosis and primary biliary
cirrhosis.
Tkeatrnent
N o therapy is known t o alter the overall course
of systemic sclerosis, but many components of
the disease may be helped significantly by speci-
fic measures. Digital ischaemiamay be helped by
electrically heated gloves and socks. Calcium-
channel blockers may help relieve Raynaud's
phenomenon. Patients with oesophageal reflux
should avoid lying flat, and treatment with
proton-pump inhibitors may be very effective.
Broad-spectrum antibiotics are helpful in treat-
ing patients with gut bacterial overgrowth and
malabsorption.
Prognosis
Severe pulmonary o r renal involvement are
poor prognostic factors, but the majority of
patients suffering from systemic sclerosis live
for many years.
Pseudoscleroderma
Scleroderma-like changes may be seen in a
number of unrelated conditions, including
porphyria cutanea tarda, carcinoid syndrome
and phenylketonuria.
There was a young belle ofold Natchez The sensation that we call itch is produced.
Whose garments were always in patches conditioned and appreciated at several levels
When comment arose in the nervous system: stimulus; mediators
On thestate afher clothes and receptors; peripheral pathways; central
She drowied:'When oh itchez,ah scratchez!' processing; interpretation. A wide variety of
(Ogden Nash. Requiem) stimuli can induce an itch, and a number o f
Pruritus means itching. Please note the correct chemicals may be involved, especially his-
spelling: it is not spelt pruritis as often appears in camine, prostaglandins and some proteinases.
student examination papers, clinical notes and However, the details remain obscure: although
referral letters! histamine can induce itch without weaiing, non-
Pruritus varies in duration, localization, and sedative antihistamines have no effect on simple
severity. Everyone hasexperienced short-term, pruritus.
localized, itch, and there is a perverse joy in hav- More complex, central mechanisms may also
ing a really good scratch. However, some indi- be important in modulating and appreciating
viduals suffer from distressing chronic irritation pruritus. Many itch-provoking stimuli induce
lasting for years. ltching may be restricted t o pain if applied at higher intensities. Indeed.
one o r more sites, o r cover virtually the whole scratchingappears to induce pain and toabolish
body surface. It may creep about, appearingtirst irritation. However, other sensory stimuli can
on an arm and later on the back, o r in more than also abolish itching, and more complex mecha-
one site simultaneously. ltching can be mild nisms have been proposed.One theory involves
o r appallingly severe, constant and distressing. a complicated filtering system controlling input
Chronic pruritus can completely ruin the qual- pathwaysto further stimuli and passing informa-
ity of life. tion on t o higher centres.
Pruritus is prominent in many skin diseases. ltching can certainly be affected by higher
Especially itchy are the eczemas, lichen pianus, centres. It is much less apparentwhen the mind
insect bites and infestations, urticaria and der- is fully occupied and much worse when bore-
matitis herpetiformis. However, the skin may dom sets in.4tressSandother psychologicalfac-
also itch when there is no rash. tors can induce orworsen pruritus.
W e d o not clearly understand why skin diseases The term'pruritus', used without qualification,
itch, and we know very little about irritation in implies that there i s itching without a primary
otherwise apparently nol-rnalskin. skin disorder However, in many instances there
l56
Causes of pruritus 157
are considerable secondary skin changes from this is scabies (see Chapter 5). A full history
scratching (e.g. excoriations, scars and and a careful examination of the skin are there-
prurigo -see below). fore important in all patients complaining of
But watch out! Subtle changes are easily itching.
obscured by scratching: a classical example of In considering causes, we shall look sepa-
rately at localized itching, generalized states
and so-called'senile' pruritus.
Localized pruritus
Localized irritation of the skin is common.The
skin may be normal, but it is more common t o
find some abnormalities.
Two very important and troublesome
forms of localized pruritus are lichen simplex
chronicus and prurigo, and anogenital pruritus.
Anogenital pruritus
Two very common (and least talked about)
forms of localized itching are pruritus vulvae
and pruritus ani. They may be encountered
together.
Treatment of perinea1 irritation depends
Pruritus ani is often attributed t o haemor-
upon the cause.
rhoids. However, although haemorrhoids and
tags are often present, their treatment alone
does not always relieve the symptoms. The
problem is also often dismissed as psycho-
logical, but only rarely is this the complete
'imimnt'pmfimsani
explanation.
* C0odhygiene.a highfibrediet.and
Anal itching may continue for years. Irritation lmeatmentwith topicalsteroids areuseful
is often spasmodic and extremely intense. The * 'IYeatingconcornitanthaemorrhoids may
majority of patients are male. reduce discharge
the skin, with a few scratch marks; o r the other features of cholestatic liver disease, espe-
skin may be covered in excoriations, scars and cially in primary biliary cirrhosis.
nodules.The skin is often dry, especially in the
elderly. Chronic renalfailure
Although there may be no identifiable under- Unfortunately the intractable itch is largely un-
lying disorder, all patients with generalized irri- affected by dialysis. Parathyroidectomy is said t o
tation should be investigated because a number help, but the benefit is short-lived and is hardly
of potentially remediable systemic disorders justified in most patients.
may be responsible.
Thyroid disease
Both thyrotoxicosis and myxoedema may pre-
sent with pruritus. In myxoedema the general
dryness of the skin may be responsible.
Haematological disorders
- * Iron deficiency
Polycythaerniarubravera
Cancers
Lymphoreticular malignancies are particularly
prone t o cause itching, but pruritus may also
Chdestpticliverdisease occur in association with a variety of carcin-
-- * Extrahepatic obstruction
Primarybiliarycirrhosis
omas. Up t o 30% of Hodgkin's disease patients
I aronicrenaltaiiure
Drugs
Various agents induce itching, but the mechan-
isms are poorly understood. Opiates appear t o
Thymiddisease act centrally and on mast cells. Oestrogens and
- * Thptoxicosis
Myxoedema
phenothiazines induce cholestasis.
1 --
Malignancy Diabetes mellitus
Lymphomas andleukaemias You may come across lists quoting diabetes as a
Carcinomas cause of itching, but we do not consider that this
is the case.
!-
1
Dmgingeetion
Especiallyopiates
Prepnancy(seeChapterl9
Psychological factors
When everything else has been excluded,
psychological factors may be considered.
The most common underlying problem is an
Haematological disorders anxiety neurosis, but patients with mono-
Chronic iron deficiency may be due t o blood delusional psychoses such as parasitophobia
loss (e.g. from menorrhagia o r agut carcinoma). also itch.These individuals, however, offer their
Many elderly patients and some vegans are iron own explanation only too readily! (see Chapter
deficient for dietary reasons. Polycythaemia 20).
rubra vera is characteristically associated with
itching triggered by bathing.
Liver disease
The itch is probably related t o bile salts in the
skin. Irritation may precede the development of
160 Chapter 18: Pruritus
Screening procedures for generalized pruri- very severe and generalized. The patients (and
tus are as follows: their carers) are often anxious and miserable,
but this is usually secondary t o the irritation
rather than a primary cause.This state is called
'senile pruritus'. It is not known what causes
ageing skin t o itch.
! * A full history and geneal examination
, * Full blood count Examination
-
* ESR (orplasmaviscosity)
Liver functiontests
* Blood redurea niuogedcreatinine
The skin is texturally either'normal' or'dry'. Ex-
coriation~,secondary eczematization and areas
of infection are common. Localized areas of
--
* Ironstudies
Serum thyroxine
Urineprotein
'eczema craquele' may develop (see Chapter 7).
* Chestx-ray neatment
Ifthese tests arenegativeinitially,mdifthe Treatment is extremely difficult. Sedative anti-
pruritus persists,repeatatintervals histamines often cause excessive drowsiness
and confusion, and topical steroids are of
limited use. If the skin is texturally 'dry', liberal
ITLeatment use of emollients may be helpful. Care has t o be
Treatment of generalized pruritus is that of its taken, however, as these agents can make both
cause. When no apparent underlying reason can the patient and their surroundings very slippery!
be found, a topical steroid and a sedative antihis- Increased frequency of washing o r the use of
tamine, such as hydroxyzine, may help. harsh soaps and detergents makes matters
worse, both by removing surface lipids and
Senile pruritus and xerosis acting as direct irritants. Soaps should therefore
ltchingwith no apparent cause is common in the be used sparingly and emollients employed
elderly. It may be mild and localized, but can be instead.
Systemic Disease
and the Skin
~
4 Diabetic dermopathy
5 Diabeticbullae
6 Xanthomas I t o be associated with diabetic rnicroangiopathy.
5 Diabetic bullae. In this uncommon blister-
ing disorder of diabetics subepidermal bullae
i
L
7 Acanthosisnigicans
8 Lipoatrophy
9 Cheiroanhropathy
~~ ~~~
I occur on the hands and feet.Their aetiology is
unknown.
6 Xanthomas. Hyperlipidaemia in uncon-
trolled diabetes may be associated with the
I Cutaneous infection. Mucosal candidiasis. development o f multiple small, yellow, erup-
particularly balanitis and vulvovaginitis, and tivexanthomas.
carbuncles, occur more frequently in diabetics. 7 Acanthosis nigricans. In association with
2 N e u r o p a t h i c ulcers. Impaired sensation. insulin resistance.
as a result of sensory neuropathy, predisposes 8 Lipoatrophy. Insulin-resistant diabetes as-
t o the development of neuropathic ulcers on sociated with partial o r genealized cutaneous
the soles ofthe feet (Fig. 19. I). lipoatrophy,
3 Necmbiosis lipoidica diabeticorum.Le-
162 Chapter 19: Systemic Disease and the Skin
9 Cheiroarthropathy. A scleroderma-like
-
thickenine of the skin o f the hands in insulin-
dependent diabetics
Thyroid disease
..
The skin is typically dry, and feels thickened due
Granuloma annulare
t o subcuraneous mucin deposition-hence the
There is no significant association between
designation myxoedema. A malar flush on an
classical granuloma annulare and diabetes, but
otherwise pale face produces what has been re-
in a much rarer generalized form of granuloma
ferred to as a'rtrawberries and cream' appear-
annulare there is an association with diabetes.
ance.There may be a yellowish tinge t o the skin,
Typically, lesions of granuloma annulare are
said t o be due t o the deposition of carotenes.
groups of firm, skin-coloured papules, often
There is often periorbital oedema Scalp hair is
arranged in rings, and commonly occurring on
diffusely thinned and there is loss of the outer
the dorsa of the hands and feet (Fig. 19.3).The
part of the eyebrows. Sirting close t o the fire t o
natural history of granuloma annulare is
keep warm may produce severe erythema ab
eventual spontaneous resolution, but persis-
igne ('granny's tarran') on the shins.
tent lesions may be treated with intralerional
triamcinolone o r cryotherapy.
Rheumatic diseases 163
Hyperthyroidism
Cutaneous changes which may accompany thy-
rotoxicosis include excessive sweating, palmar
erythema, diffuse alopecia, generalized hyper-
pigmentation and thyrotoxic acropachy (digital
clubbing). The nails may show onycholysis.
Some patienrs develop pretibial myxoedema.
which is produced by subcutaneous deposition
of excessive amounts o f mucopolysaccharide,
and ischaracterized by erythemaand thicl<ening
ofthesofttissues overthe shins and dorsaofthe
feet (Fig. 19.4).
Vitiligo may accompany autoimmune thyroid
disease, and generalized pruritus may be a fea-
tureof both hypo-and hyperthyroidism.
Adrenal disease
Cushing's syndrome
The cutaneous effects o f Cushing's syndrome
include thinning of the skin, spontaneous bruis-
ing, prominent striae on the trunl< and limbs.
diffusealopecia,acneand hirsutism. Fig. 19.4 Pretibialmyxoedema
Addison's disease
Diffuse hyperpigmentacion is the main cuta-
neous manifestation of Addison'r disease. The Rheumatic diseases
pigmentation is particularly prominent on the
buccal mucosaand in the palmarcreases.Vitiligo Gout
may also accompany autoimmune Addisonb In addition t o tophaceous deposits around af-
disease. fected joints,goutytophi may occuron theears.
164 Chapter 19: Systemic Disease and the Skin
Still's disease
Vitamin deficiency
This is a disorder of childhood, although it may
rarely occur in adults. Accompanying the
Scurvy
pyrexial episodes of Still's disease is a diffuse
The classical pictureofvitamin C (ascorbicacid)
maculopapular eruption which characteristi-
deficiency is rarely seen nowadays in developed
cally develops In the late afternoon and evening.
countries, but scurvy may be encountered in
and usually resolves by the following morning.
theelderly and in alcoholics,as a result of nutri-
Some slander-mongers claim that derma-
tional self-neglect. The typical appearance is
tologists never see this eruption because its
of perifollicular purpura, easy bruising, poor
periodicity is outside their normal working day!
wound healing and bleedinggums.
Rheumatoid arthritis
Pellagra
Dermatological features o f rheumatoid arthri-
Pellagra is the result of nicotinic acid deficiency.
tis include the following:
Classically, it has three major manifestations-
dermatitis, diarrhoea and dementia. The der-
matitis affects light-exposed areas, and there is
often a well-demarcated margin t o the affected
- Rheumatoid nodules.Subcutaneous
nodules overbany prominences,particularly
areaon the neck(Casal's necldace). Pellagramay
occur in alcoholics as a result of nutritional self-
neglect. A similar dermatitis may be provoked
on theextensoraspectofthe forearmsandthe
by isoniazid in individuals who are slow acetyla-
dorsaofthehands
* Vasculitic lesions.Digitalvasculitismay tors ofthis drug.
produce smallinfarctsamundthenail folds
(Bywaterslesions),ormoresweredigital
ulceration and even gangrene.Vasculitic Inflammatory bowel disease
lesionsmayalro m r o n thelegs,and
-
contribute to the dwelopmentoflegulcers
Fyoderma gangrenosum
* Palmarelytherna
Ulcerative colitir and Crohn's disease may be
associated with a number of mucocutaneous
manifestations includingthefollowing:
Rheumatic fever
Almost extinct in developed countries,
rheumatic fever may be accompanied by a
characterisitc eruption, erythema marginatum.
* Fyodenna gangenosum.The lesions may
be singleor multiple.Theyinitiallyresemble
Reiter's syndrome boils.whichsubsequentlybreakdown to form
Predominantly a disease of young adult males. necroticulcerswithund=-n~dpurple edges
Reiter's syndrome 1s usually precipitated by (Fig. 19.5).Fyodermagangrenosum may also
non-specific urethritis, but occasionally by accurin asrodationwith rheumatoid
bacillary dysentery. In addition t o urethritis. arthritis,myelomaand IeukaemixThe
matment ofchoiceis systemic steroids.but
conjunctivitis/uveitis and arthritis, there may
azathioprine, minocycline.clofazimineand
be an eruption which is indistinguishable from
psoriasis. O n the roles o f the feet the skin le-
sions may become extremely thickened, pro-
--
ciclosporin(cyc1osporin)may alsobeeffective
Erythemanodosum
Perianalandbuccal mucosallesions.In
ducing so-called'keratoderma blenorrhagicum'. clohns disease.analexaminationmayrevea1
The buccal mucosa may show scattered ero- fleshytags.fissuresandperianalfistulae.The
sions, and superficial circumferential erosive buccal mucosamaybeoedematousand
ulcerated,andthelipsmaybeswollenasa
changes on the penis are ~eferredtoas'circinate
resultof agranulomatouscheilitis
Sarcoidosir 165
Fig.19.6 Xanthelasma
Cutaneous metastases
Malignant tumours may metartarize t o the skin,
* Erythema nodosum.Tender.erythematous and tumours of renal, ovarian, gastrointestinal,
-
ncdulesanthelegs (seeChapterl6)
Lupuspemio.Thesldnofthenaseandears
is involved in the granulomatousprocess. and
breast and bronchial origin are those most
likely to doso (Fig. 19.8). Cutaneous metastases
becomes swollen and purplish in colour usually presentas pink nodules, and occur most
* Scarsarcoid.Sarcoidgranulamas localizein frequently on thescalpand anteriortrunk. Scalp
oldscartissue,makingthe scarsprominent metasrases may produce areas of alopecia
Fapules.nodules and plaques.Theseoften (alopecia neoplastica).
have anorange-browncolour Lymphatic extension of carcinoma t o the skin
..
may ~ r o d u c ean area of ervthematous indura-
tion resemblingcellulitis,and lknown as'carcino-
Liver disease and the skin
. .
ma ervri~eloides'.
Metastasis of ovarian or gastrointestinal car-
Changes in the skin and nails which occur in as-
cinoma via the ligamentum terer can present as
sociation with chronic liver disease include the
an umbilical nodule (Sisterjoseph's nodule).
following:
* Palmareqhema jaundice,patientswithlongstanding
-
* P~ritus:incholestaticliverdisease
SpidernaevLinasuperiorvenacaval
disnibution
cholestatic liver disease may also have
marked melaninpigmentation.Patients
-
sufferinefrom haemochramatosishave
* Xanthelasma:inp"marybiliarycirrhDsis generalized bronze-brownpigmentation
whitenails (Terry's nails) which isproducedbva combinationof iron
Pigmentary changes:in addition to and melanin
Cutaneous manifestations of systemic malignancy 167
lfyou happento have a wart on your nose or have marked psychological effects. Patients feel
forehead,you cannot help imaginingthat no one unclean, and these feelings can persist long after
in the world has anything else to do but store at the problem has been eradicated.
your warr laugh at it,and condemn you for it, There are some skin disorders which are
even though you hove discoveredAmerica. directly related t o psychological problems, and
(Fyodor Dostoevsky, The Idiot) these are described below.
Patients with skin disease who often ask'ls it
caused by nerves doctor?, are usually trying t o
establish if they can attribute their sldn condi- Dermatitis artefacta
tion to'stress'. In fact, very few skin disorders
are directly related t o psychological distur- Patients with dermatitis artefacta produce skin
bance. However, there is evidence that psoriasis lesions t o satisfy a psychological need, but what
and atopic eczema may be exacerbated by benefit they derive from their actions is usually
stress, and other conditions in which emotional not obvious. There is no rational motive for
stress has been claimed t o play a part in some their behaviour.They will vehemently deny that
cases include alopecia areata and acute the lesions are self-induced if challenged. As a
pompholyx. group they are distinct from malingerers, who
There is no doubt chat skin disease has psy- consciously imitate o r produce an illness for a
chological effects on the patient, and can signifi- deliberate end.
cantly adversely affect their quality of life. Skin Dermatitis artefacta iscommoner in women.
disease is visible t o others. it carries the raint of and most o f those affected are adolescents o r
contagion, and affected individuals often feel youngadults. However, there is a subgroup with
sdgmatized and have poor self-image and low an older age o f onset who are more likely t o be
self-esteem.They will be aware that their skin is male. Many have some connection with the
being scrutinized, and that any form of physical health professions, either directly o r via family
contact, such as shaking hands o r collecting members.
change, may provol<eapprehension in others. In Artefactual sldn lesions may be produced in a
certain ethnic groups where marriages are number of different ways including rubbing.
arranged,thepresenceofskin diseasemaycom- scratching, picking,gouging, puncturing, cutting.
promise marriage prospects, and cause con- sucking, biting, the application of heat o r caus-
siderable emotional distress. tics, o r the injection of milk, blood and faecal
Infections with ectoparasites sometimes material. Limb oedema may be simulated by the
170 Chapter 20: Skin and the Psyche
intermittent application of constricting bands treatment, relatives and friends of the patient
(Secretan's syndrome). Lesions tend t o have quite naturally rally t o their support, and may be
bizarregeometric shapes which do not conform somewhat vocal in their criticism of a seemingly
t o those seen in naturally occurring disease- inept medical profession. Other doctors caring
no dermatosis has square, rectangular o r trian- for the patient may also be convinced that their
gular lesions. Often the lesions are more disease is naturally occurring. This situation, in
numerous on the side of the body opposite the which other individuals'support' a patient with
dominant hand. If a caustic material such as an dermatitis artefacta, is known as 'folie a deux',
acid has been used t o induce lesions this may and is also encountered in delusions of parasito-
trickle off the main area of damage t o produce sis (see below).
the'drip-sign' of tell-tale streaks a t the margins. The psychopathology of patients who pro-
Even when suspected artefactual lesions duce artefactual lesions is not uniform, but in
are covered with occlusive dressings, patients some cases there is a demonstrable personality
will often manage t o insert knitting needles disorder, and in others significant depression.
under the dressings, o r push sharp instruments It requires considerable expertise t o be able
through them, in order t o continue damaging t o make a confident diagnosis of dermatitis
the skin. The history obtained from these artefacta, but even experienced dermatologists
patients is devoid of any useful information see cases in which they suspect the lesions are
about the evolution of their lesions.The impres- self-induced, but they cannot be certain. Alter-
sion conveyed is that one minute the skin was natively, there are occasional cases in which
normal, and the next it was blemished. This there is a strong suspicion of dermatitis arte-
so-called 'hollow history' is characteristic, as is facts, but the lesions are discovered t o be the
a striking complacency about what are often result of naturally occurring disease.
extremely disfiguring lesions ('10 belle indifer- Treatment is extremely difficult in many
ence'), sometimes accompanied by an enigmatic cases. Confronting the patient with the diagno-
'Mona Lisa' smile. One patient we have seen, sis is counterproductive, in that it usually
who had extensive suppuration of the left arm, produces a categorical denial that they are
probably produced by the inoculation of faeces producingthe lesions, and subsequentfailure t o
(Fig. 20. l), said about her arm 'Yes, it is rather attend for follow-up.
unpleasant isn't it. I wonder if you could arrange Strict occlusion of the traumatized areas may
for someone t o take it off'. allow healing, but the lesions will reappear as
When they see the severity of the lesions and soon as occlusive dressings are removed.
an apparent lack of progress in diagnosis and An alarming consequence of occlusion may be
the appearance of lesions elsewhere, o r the altered texture of scalp hair; the belief chat
development of other 'symptoms', as if t o they are the source of an offensive odour, usu-
compensate for inability t o reach the usual ally from the axillae o r feet. These delusional
sites. Antidepressants will help those who are beliefs o r perceptions of abnormal sensations
depressed. Psychiatric referral is often unhelp- are a consuming preoccupation for the patient.
ful and, unfortunately, many patients refuse Perinea1symptoms in men includecomplaints o f
assistance from a psychiatrist. The situation a red, burning scrotum, and the female equiva-
often remains at stalemate. As long as the lent is a burning discomfort in the vulva (vulvo-
doctor's suspicions are n o t voiced, the patient dynia). There is nothing abnormal t o see on
appears content t o continue attending for examination.
follow-up. In some cares depression is part of the
The course of this disorder is often pro- picture; others may be suffering from mono-
tracted. Recovery often has little t o do with symptomatic hypochondriacal psychosis.There
successful medical treatment, but occurs be- i s a significant risk of suicide. Management is
cause of increasingmaturity, marriage o r having d i f f i c u l ~but some patients respond t o treac-
children. ment with antidepressantsand psychotherapy.
Fig.20.2 Trichotillomania
asked to demonstrate typical sl<in'lesions', they thing. Patients often refuse psychiatric help be-
will often point t o Campbell de Morgan spots. cause they do notacceptthatthey havea mental
frecl4es or other minor blemishes. Typical illness, and cannot see how a psychiatrist could
'specimens' are presented to the doctor possibly help with what, t o them, is a physical
wrapped in pieces of paper o r adhesive tape. disorder.The neumleptic drugpimozide may be
and kept in a matchbox. These should always of benefit, if the patient can be persuaded t o
be examined under the microscope, because take it.
they just might contain parasites, but usually
they contain fragments of cotton o r sl<indebris.
I t is impossible t o persuade there patients Obsessive-compulsive habits
that parasites are not responsible fortheir con-
dition. If they are shown that their'specimens' Trichotillomania
are simple debris they remain unconvinced, and Trichotillomania means compulsive plucking of
may even suggest that the parasites are so small hair.The scalp is involved most often, but the
that an electron microscope will be required t o eyebrows and eyelashes may be affected.A mild
demonstrate them. In this situation the most form of trichotillomania may be obsel-ved in
lucid, eloquent discourse will fall upon deaf libraries, where engrossed students compul-
ears-the patient's beliefs remain unshaken, sively twist locks of hair around their fingers,
and the doctor usually retires from the conflict but they rarely pull it out unless examinations
feeling more than somewhat jaded. areapproaching!The clinical picture is of patch-
Deiusionsofparasitosismayoccurin associa- es of hair loss containing hairs ofvarying length.
tion with organic brain disease such as senile Often the crown of the head is affected, and the
dementia and cerebral arteriosclerosis, and has hairatthemargins is ofnol-mal length (Fig. 20.2).
been described in pellagra,vitamin 6,deficiency The undel-lyingscalp is usually normal, but may
and following coronary bypass surgery. The be excoriated.
term 'monosymptomatic hypochondriacal psy- Trichotillomania in childhood is often tran-
chosis' may be applied t o patients with a single. sient. However, it may bea manifestation of sig-
fixed delusion, and most pacients with delusions nificant psychopathology. particularly in adults.
of parasitosisfit into this category.
Effective treatment is very difficult, and many Neurotic excoriations
patients continue with their delusion for years. This disorder is encountered predominantly in
A confrontational approach rarely achieves any- middle-aged women.The lesions are produced
Obsessive-compulsivehabits 1 73
by picking and gouging, and are usually scattered Patients with this problem have obsessive-
over the arms, upper trunk and face. More re- compulsive personalities, and picking the skin
cent lesions are usually interspersed with scars appearst o provide relief of unconscious aggres-
from previous excoriations. Acne excoriee is a sion and tension.
variant of this condition in which minimal acne Antidepressants and supportive psychother-
lesions are repeatedly picked and gouged, leav- apy may be of benefit.
ing scars when the lesions heal.
Cutaneous Drug Reactions
Introduction -
semisyntheticpenicis and sulfonamides)
Non-stemidal anti-inflammatolydmgs
* Hypnoticr I1
The skin is one of the commonest sites for un- * Tranquillizers
wanted drug effects (a better term than 'side-
effects'), although estimates of the frequency isn't), atest may n o t be appropriate because the
vary considerably. Cutaneous drug reactions reaction is n o t t o the drug itself, but to a drug-
are probably under-reported and often go un- complex o r metabolite that is produced in viva
recognized. Note, though, that skin disorders after ingestion.
wholly unrelated t o drug ingestion can be la- The only definitive test is direct challenge
belled erroneously. It is important not t o jump with the suspected agent, but this may be im-
t o conclusions:we have seen viral infections and possible o r unethical in many circumstances.
scabies labelled as drug reactions. There are For these reasons, proving that a specific erup-
many people who state they are 'allergic t o tion was due t o a specific drug is difficult, and
penicillin', butwho are not. judgements usually have t o be made on clinical
Unfortunately there are no reliable in vitro grounds alone.
testsforestablishingthatarash is duetoadrug.
Simple in vivo tests, such as prick testing and
patch testing, havealimited placeinspecificsitu- Drug reaction patterns
ations, butusually yield no useful information.
Cutaneous drug reactions may be due t o However, all is not lost! Some drugs are much
several different mechanisms. more prone to induce cutaneous drug reac-
Note, however, that even ifthe mechanism(s) tion patterns than others. Common offenders
for a particular reaction is known (and it often include:
Drug reaction patterns 175
Furthermore, there are a number of well- and maculopapular (Fig. 2 1.1): there is often a
defined clinical drug reaction patterns. strong resemblance t o a viral exanthem. The
Some of these patterns are more drug time relationship is variable: in most instances
specific, and therefore recognition of them as the rash begins afew days after startingthe drug,
drug reactions may help t o identify the culprit. but it may begin almost immediately, o r be de-
layed for a few weeks. Exanthematic eruptions
Exanthematic eruptions usually fade a week or so after stopping the
The commonest cutaneous drug reactions are drug, but exfoliative dermatitis may develop
itchy, widespread, symmetrical, erythematous (see below and Chapter 15).
Common causes. Lanolin in creams and bandages; Common causes. Laxatives containing phenolph-
preservatives (parabens, ethylenediamine) in thalein, sulfonamides, dapsone, tetracyclines,
creams; topical anaesthetics (not lidocaine barbiturates.
Photosensitivity
There are three main types of reaction.
1
'
* Exacerbationofunderlyingdisease
Directphototoxicreaction
1 Photoalleraicreadon
1 Acne-androgenic drugs (e.g. danazol, drug provoked, and what the causitive agent
stanozoloi), oral contraceptives and cortico- might be.
steroids.
2 Porphyrias-all clinical features, including
cutaneous photosensitivity. may be worsened
by drug ingestion, particularly barbiturates and
I --
oestrogens.
Agoadhistory
3 Psoriasis-lithium, antirnaiarials. * Acarefulexamination
4 Systemic lupus erythematosus-penicillin
and sulfonamides may produce deterioration.
~
Conclusion -offenderand
Tests,where appropriate (possiblyincluding
achallenge)
If you use all the clinical informatio'n at your dis-
posal it is often possible t o determine if a rash is
Tveatrnent of Skin Disease
Ifit's dry, wet it qit's wet dry it.Congratulations, gredient (or ingredients) and a material in which
you ore now a dermatologist! (Anonymous) this is suspended-a base.These components
The above witticism is oft-quoted by non- must be compatible.There is little point in dis-
dermatologists as an assessment of the scope covering a new base which penetrates the sldn
of dermatological therapeutics. An alternative lile a hot lknife through butter if it completely
calumny relates t o a dermatologist murmuring inactivates everything suspended in it.
an unintelligible Latin name as a diagnosis and The stratum corncum forms a natural pro-
then prescribing a topical steroid, for every- tective barrier t o penetration of externally ap-
thing. Apart from being deeply offensive to plied agents. Hence, t o facilitate penetration by
sensitive skin doctors, both these quips are far a drug, this barrier function must be breached.
from the truth, as dermatologists have an enor- and this can be achieved by hydration of the
mous therapeutic armamentarium at their dis- stratum corneum-for example, penetration
posal. In days of yore, itmust beadmitted, many of a topical steroid may be markedly enhanced
of the available topical therapies resembled by occluding an area of sldn with polyrhene.
witches brews containing'Eye of newt and toe Unfortunately, if large areas of rldn are occluded
of frog, wool of bat and tongue of dog'. They in this way the amount ofsteroid absorbed may
were often cosmetically unacceptable and mal- be sufficient t o produce systemic effects. Bases
odorous-if the sldn disease did n o t render the containing urea also hydrate the stratum
patient a social pariah, the treatment could be corneum and enhance penetration of their
relied upon t o do so. However, in recent years, active ingredients. Dimethyl sulfoxide (DMSO)
topical therapies have n o t only become more is a solvent which penetrates sldn extremely
effective, but also cosmetically much more rapidly, and is used as a vehicle for the antiviral
acceptable. agent idoxuridine.
The treatment of individual disorders has
been dealt with in preceding chapters, and this
chapter is designed t o provide an overview oi Bases
the principles oftopical therapy.
An ideal topical preparation for the manage- Bases include creams, oily creams, ointments,
ment of skin disease would penetrate well, but lot~onr,gels and pastes. A cream is an oil-in-
remain localized within the skin, thereby avoid- water emulsion which is relatively non-greasy
ing potential problems from systemic effects. In and has only limited emollient activity. Creams
practice this is extremely difficult t o achieve, a l r cosmetically acceptable and can be used t o
and any agent which penetrates the stratum treat either moist o r dry skin conditions. Oily
comeum is absorbed t o some extent. creams are water-in-oil emulsions which com-
Topical preparations consist of an active in. bine good emollient properties with cosmetic
Quantities prescribed 18 1
acceptability and are tllerefore of benefit in dry oughtto be matched by the provision of precise
skin conditions. Ointments aregreasy prepara- Instructions by the doctor.Verbal instructions
tions which have emollient and occlusive arenotsufficientif multiple topical therapiesare
properties.The occlusive effect of an ointment prescribed. For example, a patient suffering
results in hydration of thestratumcorneumand from psoriasis might be given a tar shampoo, a
enhanced penetration o f the active ingredientof steroid scalp lotion.amild topical steroid cream
the ointment.The benefits of ointments are off- t o use in the flexures, and a dithranol prepara-
set by a lack of cosmetic acceptability. Oint- tionfor short contact therapy t o plaqueson the
menrsare messy and stickto clothing. If used on trunk and limbs. If the patient has only recently
the hands theytransferto everythingtouched- developed psoriasis, and is not familiar with its
an obvious disadvantage to someone employed treatment, the pmvision of multiple therapies
in clerical work, for example. Lotions are fluid without clear instructions could easily lead t o
preparations which have a cooling effect due t o confusion.
evaporation.They are useful in the management D o not expect patients who depart for work
of moist, exudative skin lesions, and also in der- at the craclc of dawn t o adhere strictly t o in-
matoses affecting the scalp. Clear, non-greasy structions to wash their hair every morning
gels are designed for use on hairy parts of the and use a topical medication twice daily Modify
body, where they are cosmetically acceptable. the treatment schedule t o suit the individual.
Pastes are powders, usually mixed with soft If you are prescribing a preparation which is
paraffin, and are protective-for example, In messy t o use andlor malodorous, warn the pa-
the prevention of maceration ofthesldn around tient about this. For example, dithranol stains
adischarging ulcer. and benzoyl peroxide bleaches, and lack of
Thechoiceofa particular base should be de- prior warning could lead t o ruined clothingand
termined by the cype o f skin problem and the bed-sheets.
sites affected. It is, for example, wholly inappro-
priate t o prescribe a steroid ointment for day-
time use on the scalp, because it is too messyA Quantities prescribed
gel o r lotion preparation should be used in-
stead. Similarly, a lotion is not the correct base It is importantwhen prescribing topical therapy
for ichthyotic skin, where an oily cream o r oint- t o consider the area t o be covered and thefre-
ment are more appropriate. qucncy of application before assessingthe quan-
Bases are mixtures of several components. tity of a topical agent required by the patient.
formulated t o provide stability and freedom For example, there is little point in prescribing
from microb~alcontamination. Random dilution a 30-g tube of an emollient t o be used over the
of a topical preparation will dilute the preserva- entire body surface after bathing-a repeat
tives in the base and significantly shorten its prescription would be required after one appli-
shelf-life. cation, because this is the approximate amount
necessary for a single application over the
whole body surfaceofan adukTopical therapies
Communication and are available in a variety of container sizes.You
patient compliance will need tochecktheavailablesizes before pre-
scribing, as they vary from product t o product.
Most non-topical medication involves popping Topical steroids, for example, may be marketed
pillsofvarious colours into the mouth atcertain in 15-. 25-, 30-, 50- or 100-g tubes, depending
times of the day, requiring a minimum of effort on the manufacturer and the steroid. Most
and only a minor feat o f memory.Topical ther- emollients are available in 50- and 100-g tubes
apy demands a great deal more of the patient. and 500-g tubs o r dispensers.
and the increased effort required ofthe patient Underprescribing and overprescribing are
182 Chapter22 TreaunentofSkin Disease
both common. One does n o t require IOOg of psoriasis of the scalp, flexures, hands and feet.
cream t o treat a small patch of eczema on the and discoid lupus erythematosus. In general,
leg-most of the tube will languish in a drawer a severe dermatosis should be treated with a
o r bathroom cabinet until its shelf-life is long potentsteroid, and a mild condition with a weak
expired, o r it may be inappropriately used by steroid. In the case o f a chronic dermatosis
another member of the family. subject t o periodic exacerbations a mild t o
moderate potency steroid can be used when
the condition i s quiescent, and a potent pre-
Topical steroids paration when it worsens.
There are regional variations in the absorp-
A t first sight the huge number of available topi- tion of topical steroids through the skin and
cal steroids is bewildering t o the uninitiated. their potential for local adverse effects. These
butwith a little knowledgeand experiencetheir variations are determined by the thickness of
use is quite straightforward. They are divided the stratum corneum, occlusion, for example in
into several groups according t o potency. the flexures where skin surfaces are in apposi-
Hydrocortisone preparations are the weakest. tion, and the vascuiarity of the area. Most facial
However, hydrocortisone in a base contain- dermatoser should only be treated with mild
ing urea, which enhances penetration of topical steroids, although a few conditions such
the stratum corneum, is moderately potent. as discoid lupus eryrhematosus will require po-
Modification of the basic steroid skeleton by tent preparations. Sldn disease affecting the ax-
fluorination (fluorinated steroids) o r esterifica- illae, groins and submammary areas should also
tion produces steroids of much greater potency betreated with mild topical steroids. Converse-
(Table 22.1). ly, dermatoses ofthe palms and soles, where the
stratum corneum is extremely thick, require
Choice of preparation potent steroids, and a greater benefit is often
The most appropriate topical steroid for a obtained if polythene occlusion is used t o en-
given situation should be determined by the hance penetration.
typeandseverity ofthecondition beingtreated, There is agreater riskofadverse systemic ef-
the sites affected,and theage ofthe patient.The fects from the use of topical steroids in c h i i d ~ m
skin disorders which are steroid responsive because of the high ratio of skin surface area t o
have been delineated in previous chapters, and bodyvolume,particularly in infants Forthis rea-
include various types of eczema, lichen planus, son mild topical steroidsshould be used in small
Mild 1% Hydroconisone
Moderately potent Clobetasonebutyrate (Eumovate)
Flurandrenolone(Haelan)
Alclometasone dipropionate(Mcdrasone)
Hydmconisone with urea (Alphadem)
Potent Betamethasonevalelate(Bemovate)
fluwinolone acetonide(Symlar)
Fluocinanide (Metosyn)
~ Hydrocortisonebutyrare(loccid)
Veypotent Clobetasolpropionatepennovate)
!
Difluconolonevalerate(NeriwneForte)
!
Topical steroids 183
children.The skin of the elderly is thin, and po- steroids on the face for lengthy periods of
tent steroids will amplify this change-their use time-often inappropriately for mild acne on
over long periods of time should therefore be the chin.The eruption consists of small papules
avoided o r carefully supervised. and pustules on an erythematous background
(Fig. 22.2).The history is virtually identical in all
Side-effects cases. Initially the mild acne appears t o improve,
Side-effects are rarely seen following the use of
mild topical steroids, but they are encountered
more frequently in association with potent
topical steroid use, although much less common-
ly than in the early years of steroid availability.
Side-effects may be divided into local, occur-
ring at the site of application of the steroid,
and systemic, resulting from percutaneous
absorption.
Local side-effects
Atrophy of the skin
Topical steroids produce dose-related thinning
of the dermis.This effect is particularly notice-
able in areas where the skin is naturally relative-
ly thin, such as the axillae, medial aspect of the
upper arm, groins and the medial aspect of the
thigh. Prominent striae may develop in these
areas (Fig. 22.1). O n the face, cutaneous thin-
ning and telangiectasia produce prominent ery-
thema. Frequent use of potent steroids on the
dorsa of the hands and the forearms results in
easy bruising, particularly in the elderly.
Perioral dermatitis
Perioral dermatitis is a condition usually seen in Fig.22.1 Prominent striae in the axilla following
young women who have used potent topical use of a potent topical steroid.
Note: Page numbers in bold refer t o tables. Page numbers in italics refer t o figures.
acantholysis 123 adrenaline (epinephrine) 132, 132 anhidrotic ectoderrnal dysplasia 106
acantholytic cells 123 adverse drug reactions see drug animal mites 47
acanthosis 68 reactions anogenital pruritus 158, 158
acanthosis nigricans 16 1, 167, 16 7 AIDS 168 anthralin (dithranol), psoriasis 73,
hyperpigmentation 1 13 albinism 1 10 74
aciclovir therapy, eczema herpeticum alginate dressing 143 antibiotics
25 allergic contact dermatitis acne vulgaris 53
acne (vulgaris) diagnosis 62-3 drug reactions 175, 175-6
age of onset and course 48 topical steroids causing 184 rosacea 56-7
assessment 54 treatment 63 antidepressants 1 7 1
clinical features 49,49-5 1,50,5 1 allergic vasculitis (leucocytoclastic antiflea agents 46
exacerbation by drugs 179 vasculitis) 144, 144-5 antifungal agents, topical 32-3,36
family 48 alopecia antihistamines
infantileljuvenile 48-9 androgenetic 1 15 senile pruritus 160
late-onset 49 cicatricial (scarring) 1 17- 18, 1 18 urticaria 132, 132
lesions 50 traction 1 17, 117 antinuclear antibodies, SLE l48
management 54 see also hair loss antiphospholipid syndrome 149
myths 54 alopecia areata 1 14, 116, 1 16- 17 antipruritic, topical 39
pathogenesis 5 1,s 1,52 alopecia totalis 1 17 antiretroviral drugs 168
psychological impact 49 alopecia universalis I 1 7, 1 18 antiseptics, topical 53
severe (acne conglobata) 50,s 1 amyloidosis 165 acne 53
severity assessment 54 anaemia, pernicious 1 13 anxiety neurosis 159
site and distribution 49 anaesthetic, local, skin biopsy 14 apocrine sweat glands 3
systemic symptoms (acne fulminans) anaesthetic lesions, leprosy 2 1 apple jelly nodules, lupus vulgaris 20
51 anagen 4,5 arrector pili muscles 3-4
treatment 53,53 anaphylactoid purpura arsenic 87
acne, secondary 54-5 (Henoch-Schonlein purpura) artefactual skin lesions 169
drug-induced 55 144-5 arthropathic psoriasis 7 5 4 7 6
oil-induced 55 anaphylaxis 176 arthropod bites 45-7
acne conglobata 50,s 1 Anderson-Fabry disease arthropods, bullae 123
acne excoriee (desjeunes filles) 56, (angiokeratoma corporis ash leaf macules 104, 1 10
173 diffusum) 107, 10 8 asteatotic eczema (eczema craquelk)
acne fulminans 5 1 androgenetic alopecia (common 66,66
acneiform disorders 55-7 balding) 1 15, 1 16 athlete's foot, (tinea pedis) 28-9,29
drug-induced 55 androgens 3 atopic eczema (dermatitis) 63-4,64
acneiform eruptions, drug-induced sebum production increase 5 complications 63
55,177 angel's kisses 98 treatment 64
acne keloidalis 56 angioedema 130-2 atopy 63
acne rosacea 56-7 hereditary 13 1, 132 atrophie blanche 142, 142
acral melanoma 9 1 treatment 132 atrophy of skin, topical steroids 183
acrochordons (skin tags) 80 angiofibromas 104 atypical mycobacteria 20
acrodermatitis continua 70 facial 105 autoantibodies
acrodermatitis enteropathica 107 angiokeratoma corporis diffusum alopecia areata 1 16
acrokeratosis verruciformis 104 (Anderson-Fabry disease) antiphospholipid syndrome 149
actinic dermatitis, chronic 139 107, l 0 8 pemphigus 123
actinic (solar) keratoses 85,86 angioma (Campbell de Morgan) 82, SLE 148
actinic prurigo 139 82 autoirnmunity, derrnatomyositis 150
acute bacterial paronychia 35 angiomatosis axillary body odour (axillary
Addison's disease 163 bacillary 168 bromhidrosis) 3
hyperpigmentation 1 13 neonatal 101 axillary bromhidrosis 3
adenoma sebaceum 104 angiomatous naevi 99, 10 1, 101 azathioprine, psoriasis 74
adrenal disease 163 angular cheilitis (perleche) 34,34 azelaic acid I I I
186 Index
Down's syndrome 40,4 1 dermatitis artefacta 169-7 1, 170 pigmentary disorders 177, 178
institutional outbreaks 4 1 dermatitis herpetiformis 126-8, 127, pruritus 159
treatment 40- 1 l28 systemic lupus erythematosus (SLE)
cryotherapy immunofluorescence 128 149
hypopigmentation I I I pathological findings 127 urticaria 176
tumours 78,78 dermatofibroma (histiocytoma) 8 1, vasculitis 176
warts 22-3 81-2 drug reactions 174, 174-9
curettage andlor cautery ('C&C'), skin dermatofibrosarcoma protuberans 92 causes 174
tumours 77.77-8 dermatoglyphics 6, 7 exacerbation of pre-existing disease
Gushing's syndrome 54, 163 dermatological assessment I I 178-9
steroid side-effects 184 dermatological history 10, 10 exanthematic eruptions 175, 175-6
cutaneous herpes simplex 25 dermatological non-disease fixed eruptions 176-7, 177
cutaneous metastases 166-8,16 7, (dysmorphophobia) 17 1 lichenoid (lichen planus-like) 177
168 dermatological pathomimicry 1 7 1 light-induced 139
cutaneous tuberculosis 19 dermatomyositis 150-2, 15 1 patterns 174-9, 175
cuticle muscles 1 5 1-2 Duplex sonography, venous leg ulcers
hair 4 skin changes 150, 150 142
nail 5-6 dermatophyte infections 28-33 dysbetalipoproteinaemia 165
cyclosporin (ciclosporin) 74 cattle ringworm 3 1,33 dysmorphophobia (dermatological
cyproterone acetate 53 diagnosis 32 non-disease) 17 1
cyst(s) 'ide' reaction 32 dysplasia 83
acne vulgaris 50 kerion 3 1,32 dysplasticlmalignanttumours, of skin
epidermal 81 mycetoma (Madura foot) 33 dermal 92-3
epidermoid 8 1 treatment 32-3 Kaposi's sarcoma 92-3
keratin (milia) 8 1,8 1 see also entries beginningtinea lymphomas 92-3
myxoid 120,12 1 dermatophytes 28 epidermal 84-7
pilar (trichilemmal) 8 1 dermatosis 62 actinic (solar) keratoses 85,86
cystic fibrosis 3 juvenile plantar 66,66 basal cell see basal cell carcinoma
cytotoxic drugs dermis 6 (rodent ulcer)
adverse effects 74 tumours involving 78 prevention 9 1-2
psoriasis 74 dermoepidermal junction 96 squamous cell see squamous cell
dermographism 1 3 1, 13 1 carcinoma (SCC)
danazol 132 desmosomes 2 melanocytic tumours 89-9 1
dapsone 127 diabetes mellitus 16 1, 16 1-2 lentigo maligna (Hutchinson's
Darier's disease (keratosis follicularis) candidal balanitis/vulvovaginitis 35, malignant freckle) 89,89,90
1 04,104 16 1 melanoma see melanoma,
delayed hypersensitivity 60, 176 pruritus 159 malignant (MM)
delusions of parasitosis (parasitosis) diabetic bullae 16 1 prevention 9 1-2
171-2 diabetic dermopathy 16 1 dysplastic naevus 98
dendritic cells 2 diagnosis of skin disease 9-1 6 dysplastic skin changes 8 3 4
depigmentation l 10, l l I dermatological history 10
dermabrasion, acne vulgaris 53 diagnostic process 9-1 0 eccrine sweat glands 3
dermal papillae 6 value 9,9 absence 106
dermal tumours see also examination; investigation ectodermal dysplasia, anhidrotic
benign 8 1-3 dimethyl sulfoxide (DMSO) 180 l06
see also benign tumours dioxin exposure 55 ectoparasite infections 37-47
malignant 92-3 discoid eczema 65 crusted (Norwegian) scabies 40- 1,
see also dysplasticlmalignant discoid lupus erythematosus 1 18, 41
tumours 149,149-50 papular urticaria 45-7
dermatitis disseminated zoster 26 pediculosis 4 1-5
allergic contact see allergic contact dithranol (anthralin), psoriasis 73,74 psychological effects 169
dermatitis Down's syndrome 108 scabies 3740,39
atopic see atopic eczema crusted (Norwegian) scabies 40,4 1 see also individual infections
(dermatitis) drug-induced conditions eczema 58-66,59
chronic actinic 139 acne 55 asteatotic (eczema craquele) 66,66
exfoliative see erythroderma acneiform lesions 55, 177 atopic see atopic eczema
hair dye 62 blisters 123 (dermatitis)
nickel 60-1,6 1 bullous disorders 123, 177, 177 classification 58,59
occupational contact 62 contact dermatitis 176, 176 clinical features 58,59
occupational irritant 60 eczema 176 discoid 65
in pellagra 164 erythema multiforme 177 drug-induced 176
perioral 57 erythroderma (exfoliative endogenous 5 8 , 6 3 4
topical steroids 183, 1 83-4 dermatitis) 176 palms and soles 65-6
plant 62 hair abnormalities l 7 7 exogenous 58,5943
primary irritant 59-60,60 hair loss I 16 lichenified 58,59
rubber 61 hypopigmentation I I I photosensitive 139
topical medicaments causing 62 lupus erythematosus 178 varicose 65,142
see also eczema photosensitivity 178, 178, 178 see also dermatitis
188 Index