WHAT IS THE DIAGNOSIS?

Dermal cylindroma is a benign

neoplasm of the eccrine sweat
glands, which presents in the head
and neck area in majority of cases. In
its most florid presentation, the
entire scalp can be involved, leading
to the descriptive label turban tumor.
They most commonly occur on the
head and neck as solitary or multiple
tumors. Solitary cylindromas occur
sporadically and typically are not
inherited. Multiple tumors are
observed in an autosomal dominantly

BCCS,PALMER PITS,JAW
CYSTS

Diagnosis of GORLIN SYNDROME is made by having 2 major criteria or 1

major and 2 minor criteria. [2]
The major criteria consist of the following:
more than 2 BCCs or 1 BCC in a person younger than 20years;
odontogenic keratocysts of the jaw
3 or more palmar or plantar pits
ectopic calcification or early (<20years) calcification of the falx cerebri
bifid, fused, or splayed ribs
first-degree relative with NBCCS.
The minor criteria include the following:
macrocephaly.
congenital malformations, such as cleft lip or palate, frontal bossing, eye
anomaly (cataract, coloboma, microphtalmia, nystagmus).
other skeletal abnormalities, such as Sprengel deformity, pectus deformity,
polydactyly, syndactyly or hypertelorism.
radiologic abnormalities, such as bridging of the sella turcica, vertebral
anomalies, modeling defects or flame-shaped lucencies of hands and feet.
ovarian and cardio fibroma or medulloblastoma (the latter is generally
found in children below the age of two).

What is the diagnosis?

Bullous pemphigoid may present with several distinct clinical presentations,

as follows:
Generalized bullous form: The most common presentation; tense bullae arise
on any part of the skin surface, with a predilection for the flexural areas of
the skin
Vesicular form: Less common than the generalized bullous type; manifests as
groups of small, tense blisters, often on an urticarial or erythematous base
Vegetative form: Very uncommon, with vegetating plaques in intertriginous
areas of the skin, such as the axillae, neck, groin, and inframammary areas
Generalized erythroderma form: This rare presentation can resemble
psoriasis, generalized atopic dermatitis, or other skin conditions
characterized by an exfoliative erythroderma
Urticarial form: Some patients with bullous pemphigoid initially present with
persistent urticarial lesions that subsequently convert to bullous eruptions; in
some patients, urticarial lesions are the sole manifestations of the disease
Nodular form: This rare form, termed pemphigoid nodularis, has clinical
features that resemble prurigo nodularis, with blisters arising on normalappearing or nodular lesional skin
Acral form: In childhood-onset bullous pemphigoid associated with

What is the diagnosis?

Pseudoxanthoma elasticum (PXE), also known

as GrnbladStrandberg syndrome,[1] is a
genetic disease that causes fragmentation and
mineralization of elastic fibers in the skin and
eyes, and later in blood vessels in the form of
premature atherosclerosis.[2] PXE is caused by
autosomal recessive mutations in the ABCC6
gene on the short arm of chromosome 16
(16p13.1).[2][3]
They consist of small, yellowish bumps in rows or a lacy
pattern, which may join to make large patches. The skin is
soft, lax and slightly wrinkled. The patches may be slightly
pebbly in appearance, which has been described as
cobblestoned.
The common sites affected are the sides of the neck,

What is the diagnosis?

Chronic and progressive
Typically affects elderly women
Increased prevalence of HLA-B12

Oral mucosal lesions in most cases

Skin lesions are less common

Cicatricial pemphigoid is a systemic autoimmune disease with

both ocular and extraocular manifestations. Its effect on the
eyes is characterized by chronic cicatrizing conjunctivitis and
progressive conjunctival sub-epithelial fibrosis with resultant
fornix foreshortening, symblepharon formation, trichiasis,
meibomian, and lacrimal duct obstruction resulting in sicca
syndrome, ocular surface keratinization, and eventually
blindness. Extraocular manifestations include stricture formation
of the esophagus, trachea, anus, or vagina. OCP is characterized
by deposition of immunoreactants at the conjunctival basement
membrane zone, initiating a type II hypersensitivity reaction.
Demonstration
of this deposition
can known
be done as
using
Cicatricial pemphigoid
(also
"Mucous
immunofluorescence or immunoperoxidase technique.

Membrane Pemphigoid", "MMP", "Benign mucosal

pemphigoid," "Benign mucous membrane
pemphigoid,"[1] "Ocular pemphigus,"[1] and "Scarring
pemphigoid"[1]) is a rare chronic autoimmune
subepithelial blistering disease characterized by
erosive skin lesions of the mucous membranes and
skin that results in scarring of at least some sites of
involvement.[2]:581

Recurrent,child,pruritis,bullous
eruption,diagnosis?

 chronic bullous disease of childhood (CBDC) is a nonhereditary,

autoimmune
subepidermal bullous disease, characterized by the presence
of continuous linear deposits of IgA autoantibodies
along the basement membrane zone. ,CBDC is characterized
by a clinical polymorphism in each patient, with the cluster of
jewels pattern being the most typical clinical lesion. For the
establishment of CBDC diagnosis, combination of
histology and immunofluorescence studies are of utmost
importance. The majority of patients respond to dapsone,
sulfapiridine or systemic steroids in cases of widespread
disease. CBDC tends to resolve spontaneously within
several months to 5 years after its onset.

Linear IgA disease is usually idiopathic i.e. it

arises spontaneously. However, it sometimes
follows infection and is rarely caused by drug
allergy. Vancomycin is the most frequently
associated drug, although diclofenac,
captopril, cotrimoxazole, amiodarone,
ciclosporin, glibencamide, lithium, penicillin,
cefamandole, phenytoin, sodium
hypochlorite, and somatostatin have been
implicated in case reports. Drug-induced
disease resolves with withdrawal of the
offending agent. Linear IgA disease has also
been rarely associated with lymphoma,
haematological conditions, rheumatological

Genetic,blistering
disorder,diagnosis?

What is the diagnosis?

There is subepidermal cleft

Papillary tip abscess

Direct immunoflourence shows granular IGA

deposition

Dermatitis herpatiformis
During disease
Related to coelic
disease
Involves IG A
antibodies
There is intolerence to
gliaden fraction of
gluten
2/3 are males
Association with hla
dq2dq8

Pruritis severe
Vesicles and excoriated
papules
Symmetrical distribution
Scalp,shoulders,buttock,el
bows and knees
Hyper or
hypopigmentation is seen
Small bowel villous atrophy
Dapsone,sulphapyridine
Gluten free diet

What is the
diagnosis?

TREATMENT
Systemic steroid ;
2 to 3 mg/kg of prednisolone until cessation of new blister formation and disappearance of Nikolsky sign.

Concomitant Immunosuppressive Therapy(steroid sparing agents)

such as Azathioprine , 23 mg/kg
Methotrexate , either orally or IM at doses of 25 to 35 mg/week.
cyclophosphamide or mycophenylate mofetil
High-dose intravenous immunoglobulin (HIVIg) ;
(2 g/kg every 34 weeks) may help gain quick control whilst waiting for other
drugs to work.
Rituximab ( Anti-CD20 monoclonal antibody) has been reported to help
multidrug resistance, IV , once a week for 4 weeks.
Rx need regular follow up and is usually prolong
Dosage should be dropped only when new blisters stop appearing

WHAT IS THE DIAGNOSIS?

Acute facial paralysis that occurs in association with herpetic

blisters of the skin of the ear canal, auricle, or both is referred to
as the Ramsay Hunt syndrome, or herpes zoster oticus.

9 months history

Biopsy shows
tuberculoid
granulomatous
inflamation

Give diagnosis
Name drugs used

Diseases with tuberculoid

reaction

Tuberculosis
Tuberculids
Leprosy
Leishmaniasis
Late syphilis
Lupus miliaris
desseminatus facie
Crohns disease
Rosacea
GRANULOMATOUS
CHEILITIS

WHAT IS THE DIAGNOSIS?

GRANULOMATOUS CHIELITIS
RECURRENT FACIAL
PARALYSIS
SWELLING OF THE
LIPS
SOME TIMES OF
THE FACE
THIS MAY BE
FAMILIAL
ETIOLOGY IS
UNKNOWN

HISTOPATHOLOGY
SHOWS
TUBERCULOID
GRANULOMATOUS
INFLAMATION

WHAT IS THE DIAGNOSIS?

MAFFUCI SYNDROME
MULTIPLE HAEMANGIOMAS
MULTIPLE ENCHONDROMAS
RISK OF DEVELOPING PANCREATIC
TUMOURS,OVARION TUMOURS,CNS
TUMOURS

pruritic
skin
eruption

Name 3
diseases
presentin
g in such
fashion

Diseases with linear

presentation

PSORIASIS
LICHEN PLANUS
LICHEN STRIATUS
LINEAR EPIDERMAL NEVUS
NEVOID LENTIGINOSIS
POROKERATOSIS
MORPHEA
CERTAIN INFECTIONS

WHAT IS THE DIAGNOSIS?

TELENGIACTASIS
COMMON IN JEWS
OF EASTERN
EUROPIAN ORIGIN
EARS CAN BE
PROMINENT
PHOTOSENSITIVITY
SHORT STATURE
AUTOSOMAL
RECESSIVE

ROTHMAN THOMSON
SYNDROME
AUTOSOMAL
RECESSIVE
POIKILODERMA ON
THE
CHEEKS.,HANDS,B
UTTOCKS,
30% CASES SHOW
HYPOGONADISM,
PHOTOSENSITIVITY

SHORT STATURE
SPARSNESS OF
EYEBROWS,EYLASHES
,
ALOPECIA OF SCALP
CONGENITAL BONE
DEFECTS
SQUAMOUS CELL
CARCINOMA,BASAL
CELL CARCINOMA MAY
DEVELOP

BLOOM SYNDROME
Autosomal recessive
disorder characterized
by telangiectases
photosensitivity
, SHORT STAURE
immunodeficiency,
increased susceptibility
to neoplasms .
HIGH PITCHED VOICE.
Prominent nose and ears
hypogonadism]

Psoriasiform infiltrated
plaques
Biopsy shows
granulomatous
inlammation
with many
eosinophils

What is the diagnosis?

What is the diagnosis?

Granuloma annulare (GA)

is a benign inflammatory,
self-limiting
granulomatous
dermatoses that is seen
The lesions
could be related to insect
in both adults and
bites, children
sun exposure, viral infections,

diabetes, thyroiditis, immunoglobulinmediated vasculitis,and certain

medications such as antibiotics,
antiinflammatory agents and oral
contraceptives.
Cases have also been reported in
patients with AIDS, sarcoidosis,
hepatitis C infection, Hodgkin's and
non Hodgkin's lymphoma, metastatic

Clinical variants:
(1) Localized - Children & young adults. Papules &
annular or arciform plaques.
Site: Hands, feet, arms & legs.
(2) Generalized - Middle aged and elderly patients.
Multiple macules, papules or nodules.
Site: Trunk & limbs.
(3) Perforating - Middle aged & elderly females.
Papules & annular plaques. Scaling & crusting with
central umbilication may be present.
Site: Dorsa of the hands and feet.
(4) Subcutaneous or deep form - Children & young
adults. Solitary or multiple nodules (a few
millimeters to several centimeters in size).
Site: Lower extremities, dorsa of the hands & feet,
buttock and scalp.

What is the diagnosis?

What is the diagnosis?

Acanthosis Nigricans
Seen in situations of insulin resistance
Besides in DM, also seen in the following:

Carcinomas, especially of the stomach

Secondary to meds (nicotinic acid, estrogen, or
corticosteroids)
Pineal tumors
Other endocrine syndromes (PCOS, acromegaly,
Cushings disease, hypothyroidism)
Obesity

Pathogenesis

According to Cruz (12) , it may be related to insulin

binding insulin-like growth factor receptors on
keratinocytes and dermal fibroblasts, thus stimulating
growth.

What is the diagnosis?

WOOD LAMP
EXAMINATION
SHOWS CORAL
RED FLORESCENCE

What is the diagnosis?

She has developed

hyperpigmented ,
firm plaques on
both lower legs

Hypothyroidism and the

Skin

Skin changes in hypothyroidism reflect a hypometabolic

state and subsequent reduced core body temperature
results in cutaneous vasoconstriction. (14)
Skin is cool, dry, and pale.
Pallor results from cutaneous vasoconstriction and increased
deposition of water and mucopolysaccharides in the dermis,
which alter the refraction of light

Hypohydrosis may lead to palmoplantar keratoderma

Carotenemia (from decreased hepatic conversion of beta
carotene to Vit A) gives skin yellowish hue (14, 17)
Hair: dry, brittle, coarse; partial alopecia
Loss of hair from lateral 1/3 of eyebrows

Gottron papules
What is the diagnosis?

Criteria for diagnosis: Dermatomyositis

1. Symmetrical limb girdle weakness
2. Muscle biopsy evidence of inflammatory myonecrosis.
3. Elevated muscle enzymes (CPK, Aldolase, LDH).
4. Characteristic EMG features
5. Dermatological features
1.

Heliotrope with periorbital edema

2.

Gottrons sign - scaly dermatitis over dorsum of hands, extensor joint

surfaces, face, upper torso
Definite :
Probable :
Possible :

3 criteria plus rash

2 criteria plus rash
1 criteria plus rash

Dermatomyositis
Heliotrope erythema:

periorbital purple/erythema and

edema
erythematous
Gottrons
sign: scaling macules and papules dorsa

of the knuckles and elbows

Periungual erythema

WHAT IS THE DIAGNOSIS?

LIVEDO RETICULARIS
MOTLED DISCOLOURATION DUE TO
CAPPILARY DILATATION AND
STAGNATION OF BLOOD WITHIN IT.
CUTANEOUS DISCOLORATION
SURROUNDING PALE CENTRAL AREAS.
IDEOPATHIC
VASCULITIS.SLE,DERMATOMYOSITIS,AN
TIPHOSPHOLIPID SYNDROME
RHEUMATOID ARTHRITIS,TB.

What is the diagnosis?

What is the diagnosis?

What is the diagnosis?

WHAT IS THE DIAGNOSIS?

Child
Fever with skin
peeling
oral mucosa not
involved
PROGNOSIS GOOD

Stevens-Johnson syndrome
Acute, and self-limiting
Hypersensitivity to drugs or infection
Typically affects young men

Lesions of oral mucosa

Vesciculobullous haemorrh
Maculopapules which
and lips
necrotic lesions
may develop intoand
target
lesions

Give the diagnosis?

Give DDS

What is the diagnosis?this is some

test

Tuberous sclerosis

Seizures
Mental retardation
Slow growth
AD
Angiofibromas
Periungual fibroma
Shagreen patgh
Ash leaf spots

FAIR COLOUR OUTDOOR

WORKER
COMPLAINS OF
PAPULAR SKIN
ERUPTION
BIOPSY SHOWS
HOMOGENEOUS
EOSINOPHYLIC
MATERIAL IN
PAPILLARY DERMIS
NAME THE DISEASE

What is the diagnosis?

Ehlers danlos syndrome

Bazexs Syndrome

Violaceous, symmetric papulosquamous plaques on the acral

surfaces of ears, nose, hands, and feet

BAZEX SYNDROME
It is also known as acrokeratosis
neoplastica
Nail dystrophy with painful
paronychia
Scaly eruption on the face
Keratoderma of hands and
feet,honey comb appearance
Squamous ca. of upper res. Tract
upper GIT tract
More common in males

Hand-Foot-Mouth:
Enanthem

Hand-Foot-Mouth:
Exanthem

Hand-Foot-Mouth Disease
Hand-foot-and-mouth disease (HFMD) is a
viral illness with a distinct clinical
presentation of oral and characteristic
distal extremity lesions
Usually Coxsackie A16
At risk: preschool children
Incubation period
Prodrome: 1-2 days before rash
Low grade fever, anorexia, malaise, sore mouth

Case of the week

Case of the week

Generalized pruritus
without an eruption
Causes:

Idiopathic (senile)

Iron deficiency

Liver disease

Malignancy (e.g. Hodgkins lymphoma)

Neurological disorders

Polycythemia

Renal failure

Thyroid dysfunction

immunoflorescence
Used to find the presence of antibodiies
attached to the antigens in tissues
Flourescene linked antibodies are used
Fluorescin isothiocyanate is used
Direct immunoflorecence
Indirect immunoflorescence
Complement indirect immunoflouresence
Salt split technique
Liquid nitogen,slid carbone dioxide,hexane
bath or Michal solution