DOI:10.1542/pir.

13-4-130
1992;13;130-137 Pediatr. Rev.
Shelley Lanzkowsky, Leora Lanzkowsky and Philip Lanzkowsky
Henoch-Schoenlein Purpura
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Henoch-Schoenlein Purpura
Shelley Lanzkowsky, MD,* Leora Lanzkowsky, MD,** and
Philip Lanzkowsky, MD***
FOCUS QUESTIONS
1. W hat is our understanding of the
pathophysiology of Henoch.
Schoenlein purpura (HSP)?
2. W hat are the cardinal clinical fea-
tures of HSP?
3. W hich clinical features offer sub-
stantial threat to survival or full
recovery from HSP?
4. W hat clinical or laboratory obser-
vations are required for or appro-
pilate to m onitoring the course of
HSP?
5. W hat therapeutic measures are
available for HSP, and what clini-
cal features direct their choice and
use?
Altending Pediatrician, Childrens Special-
ized Hospital, M ountainside, New Jersey.
5 Dep tmerJ of Radiology, Long Island
Jewish M edical Center, New Hyde Park, New
York.
Department of Pediatrics;
Chief-of-Staff, Schneider Childrens Hospital,
Long Island Jewish M edical Center; Profes-
sor of Pediatrics, Albert Einstein College of
M edicine. Address reprint requests to: P.
Lanzkowsky, Department of Pediatrics,
Schneider Childrens Hospital, Long Island
Jewish M edical Center, New Hyde Park, NY
11042.
130 Pediatrics in Review VoL 13 No. 4 April 1992
ARTICLE
The distinctive syndrome of Henoch-
Schoenlein purpura (HSP) was first
described by Heberden before 1800,
and in 1808 English physician Robert
W illan described a patient who had
swollen, painful joints and a rash.
The syndrome owes its name to
two German physicians. In 1837,
Johan Schoenlein described several
cases of purpura associated with ar-
thritis, which he termed peliosis
rheumatica. In 1868, Edouard
Henoch pointed out that the term
peliosis rheumatica was inappro-
priate and restrictive because patients
who had urticarial purpura and acute
arthritis also manifested gastrointes-
tinal symptoms, such as vomiting,
colicky abdominal pain, and melena.
He described four children who had
purpura, colicky abdominal pain, gastro-
intestinal hemorrhage, and joint pain
as well as renal involvement) Since
these first descriptions, HSP also has
been referred to as anaphylactoid, al-
lergic, or rheumatoid purpura; leuko-
cytoclastic vasculitis; and allergic
1.2
Epidemiology
Seventy-five percent of all cases of
HSP occur in children between 2 and
1 1 years of age, with younger chil-
dren rarely affected. Children
younger than 2 y experience a milder
course of illness, with less frequent
renal and gastrointestinal manifesta-
tions.3 HSP is exceedingly rare in
adults, among whom other causes of
vasculitis should be considered more
likely.
The incidence of HSP is greater in
males, with a male-to-female ratio of
1.5 to 2.0:1. In adults, the sex ratio
approaches 1 : 1. There appears to be
a seasonal incidence, with more
cases in the spring and autumn
months. Recent reports indicate that
HSP may be less common in black
than white children.4
Etiology
The etiology of HSP-like that of a
similar vasculitis, Kawasaki dis-
ease-is unresolved. In 1914, W il-
ham Osler hypothesized an allergic
basis for the illness.5 Seventy-five
percent of patients give a history of
upper respiratory tract infection pre-
ceding the onset of this syndrome.
Streptococcal pharyngitis once was
thought to play a large role in patho-
genesis, but studies have shown that
the incidence of elevated streptococ-
cal antibody titer in patients who
have HSP is no higher than that of
controls matched for age.3
Other infectious agents reported to
have preceded HSP have included
those due to Yersinia, Legionella,
parvovirus, adenovirus, M ycoplasma,
Epstein-Barr virus, and varicella.56
Cases also have been reported fol-
lowing vaccination against typhoid,
paratyphoid A and B, measles, chol-
era, and yellow fever. In addition,
the illness has been linked to such al-
lergens as drugs (penicillin, ampicil-
lin, erythromycin, quinidine, quinine)
and foods, to exposure to cold, and
to insect bites. No precise etiology
has been established; seasonal varia-
tion in incidence has been noted, but
no clear-cut pattern is apparent.
Pathogenesis
HSP is thought to be an IgA-m e-
diated vasculitis in small vessels of
involved organs. IgA levels become
elevated either because of increased
production or decreased renal clear-
ance of IgA. It is postulated that an
unknown antigenic stimulant causes
elevated IgA, activating pathways
leading to necrotizing vasculitis.
Lymphocytes, neutrophils, circulating
immune complexes, IgA, C3, and fi-
brin infiltrate systemic blood
Despite differences in clinical pre-
sentation, the renal lesion of HSP is
indistinguishable histopathologically
from that of IgA nephropathy (Berger
disease). Renal involvement occurs
in 20% to 50% of patients who have
HSP; approxim ately 30% of patients
who have Berger disease have extra-
renal manifestations of rash and ar-
thralgia. Both diseases result in
elevated IgA production, increased
circulating immune complexes of
IgA, and decreased Fc receptor-me-
diated immune clearance. The dis-
eases have identical renal histology,
and the dermal vessels in both ill-
nesses show IgA immunofluores-
cence. Patients who have HSP are, in
general, younger than those who
have IgA nephropathy, but the age
distribution overlaps. Both diseases
carry the risk of progression to renal
insufficiency, and recurrent disease
develops in both following renal
transplantation. The similarity of fea-
tures in these two illnesses can be
explained by a similar pathogenesis
with different expressions in the
hosts. In the immature host, the cir-
culating immune complexes may
generate systemic HSP, whereas the
older host may develop only renal
manifestations of the illness.4
Clinical Manifestations
The hallm arks of HSP are a non-
throm bocytopenic purpuric rash, ab-
dom inal pain, arthritis, and nephritis.
The illness m ay follow various clini-
cal courses, however. The character-
istic symptoms may appear in any
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Ta b l# {1 4 9 } 1 . P re s e n tIn g S y m p to m s a n d S ig n s In
S c h o n le ln P u rp u ra
H. n o c h -1
JI___J
ORGAN S YMP TOMS AND S IGNS
Cardiac M yocardial infarction (coronary vasculitis)
Central nervous system Apathy
Headache
Hyperactivity
Irritability
Rare symptoms and signs (see Table 2)
Somnolence
Gastrointestinal tract Acute appendicitis
Colicky abdominal pain
Gastrointestinal hemorrhage
Guaiac-positive stools
Hematemesis
Hepatomegaly
Hydrops of gallbladder
Ileus
Intussusception (ileoileal in 65% of cases)
M elena
Pancreatitis
Vomiting
Genitourinary tract Hematuria and proteinuria
M icroscopic hematuria
Nephritic and nephrotic signs
Nephritic signs
Scrotal involvement
M usculoskeletal Arthritis/arthralgia
Intramuscular hemorrhage
Non-specific Fever
Preceding infection
Pulmonary System Pulmonary hemorrhage
Serosanguinous pleural effusion
Skin Erythematous maculopapules
Petechiae
Purpura
Subcutaneous edema (scalp, extremities)
Urticaria
Pediatrics in R eview V oL 13 N o. 4 A pril 1992
3
1 - -
H

n o c h
HEMATOLOGY I
-Schosnl&n Purpura I
. -- - -
order; accordingly, there may be a
variety of clinical presentations. The
presenting symptoms and signs in
HSP are listed in Table 1.
SKIN
The typical rash occurs in 100% of
cases and is the presenting feature in
50% of cases. Lesions appear on the
lower extremities and buttocks, but
may involve upper extremities, face,
and trunk. The classic lesions consist
of urticarial wheals, erythematous
maculopapules similar to those found
in erythema multiforme, or larger
palpable ecchymotic-looking lesions
(Figs 1-3). In addition to these char-
acteristic lesions, various patterns of
erythema multiforme may occur. Le-
sions initially blanch on pressure, but
later lose this feature. Petechiae or
purpuric lesions occur, but there is
no associated thrombocytopenia. The
purpuric areas evolve from red to
purple, becoming rust-colored with a
brownish hue, and eventually fade.
New crops may arise, giving a poly-
morphic appearance. Occasionally
the rash is pruritic. The rash can per-
sist for weeks, be transient, or recur.
Deviations from this classic pre-
sentation do occur. Vesicular erup-
tions have been described, as have
erythema multiforme with central
hemorrhage or ulceration and forma-
tion of bullae.7 Furthermore, the typ-
ical distribution over the buttocks
may be completely absent, and HSP
has been described with orofacial le-
sions alone.
Angioedema of the scalp and of
the extremities occurs in 20% and
46% of cases, respectively. Angio-
edema is nonpitting; may involve
eyelids, lips, dorsa of the hands and
feet, back, and perineum; and may
be especially striking in children
younger than 3 y. Rarely, an entire
limb may be transiently swollen and
tender.
JOINTS
Arthralgia, arthritis, or both occur in
68% to 75% of cases. They precede
the rash and are the first signs of
HSP in approximately 25% of pa-
tients.3 Joint involvement tends to be
periarticular, usually without bleed-
ing or effusion into joints. Affected
joints may be swollen, tender, and
painful on motion. The tenderness
and swelling are mainly periarticular.
Ankles and knees are the most com-
monly affected joints, whereas the
fingers and wrists usually are not in-
volved. Joint involvement is transient
or may recur during active disease,
but leaves no permanent deformity.
GASTROINTESTINAL TRACT
The most frequent symptom of HSP
following rash and joint pain is ab-
dominal pain, which occurs in ap-
proximately 35% to 85% of cases. In
the majority of cases, cutaneous
manifestations of HSP precede ab-
dominal symptoms, but visceral man-
ifestations precede the rash in 14% of
cases. The most common complaint
is colicky abdominal pain, which
may be severe and associated with
vomiting. Stools show gross or oc-
cult blood in more than half of the
cases, and hematemesis may occur.
Abdominal pain associated with HSP
is occasionally difficult to distinguish
from other causes of abdominal pain
and may mimic that of an intraab-
dominal emergency.
M ost cases of abdominal pain are
due to submucosal and intramural cx-
travasation of fluid and blood into
the intestinal wall, which may lead to
localized ulceration of the mucosa
(causing guaiac-positive stools) and
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HEMATOLOGY
H#{ 149} noch-Schoinleln P u rp u ra
FIGURE!. Fairly we l l defined
e ythematous, urticarial lesions with
some slight petechial lesions on posterior
aspect of lower extremity of an individual
wh o has Henoch-Schoenlein purpura.
Typical location for these lesions.
FIGURE 2. F airly w ell defined
etythem atous, urticarial lesions w ith
som e slight petechial lesions on low er leg
and ankle of an individual who has
H enoch-Schoenlein puipura. Typical
location for these lesions.
FIGURE 3. An e,ythematous, urticaria4
slightly edematous lesion that is ill
defined on helix and antihelix of ear of
an individual w ho has Henoch-
Schoenlein purpura. N ot a com m on
location for this condition.
132 Pediatrics in Review V oL 13 N o. 4 A pril 1992
may be associated with diffuse arte-
rial inflammation and fibrinoid necro-
sis. This is due to mesenteric
vasculitis.
The radiographic manifestations of
HSP are nonspecific, and other
causes of intramural bleeding (var-
ious coagulopathies or other forms of
vasculitis) and inflammatory changes
(such as inflammatory bowel disease)
may appear similar. Clinical correla-
tion is extremely important.
The following six gastrointestinal
radiographic findings are consistent
with a diagnosis of HSP8: 1) uniform
regular thickening of small bowel
wall folds, causing parallel, symme-
tric, spike-like configurations simu-
lating a stack of coins or picket
fence, most striking in the jejunum
(Figs 4 and 5); 2) blurring of the
sharpness and symmetry of the small
bowel wall folds, due to edema of
the small bowel and increased secre-
tions; 3) thumbprinting and scallop-
ing due to local hemorrhage of bowel
wall (Fig 6); 4) filling defects in
bowel wall due to mucosal edema
and small areas of dilatation as a re-
suit of vascular occlusion in the sub-
mucosa; 5) intramural or extramural
masses, flattening of the folds on the
mesenteric side, and separation and
uncoupling of the bowel loops, rep-
resenting bleeding into the mesentry;
6) coil-spring appearance of small
bowel intussusception (Fig 4). These
nonspecific radiographic findings all
are transient and usually resolve
within 4 to 6 wk without any residual
defects.
A sudden onset of or increase in
abdominal pain associated with HSP
may be secondary to intussusception,
bowel infarction, bowel perforation,
pancreatitis, or hydrops of the gall
bladder.
I ntussusception occurs in 2% to
3% of patients, but infarction and
perforation of the bowel are uncom-
mon. The incidence of abdominal
conditions requiring surgery in asso-
ciation with HSP is 2% to 6%. M ost
surgical complications occur after the
appearance of purpura. Although ab-
dominal complaints that appear prior
to the rash may be severe, they are
unlikely to be due to infarction ne-
crosis or perforation of the gut.9
I ntussusception associated with
HSP is often the result of a submu-
cosal hematoma and is ileoileai in
65% of cases, whereas 95% of cases
of intussusception in the population
at large are ileocolic. A barium
enema may miss the iieoileal intus-
susception; accordingly, ultrasonogra-
phy is used to diagnose intussuscep-
tion in HSP both because it is nonin-
vasive and because it identifies intus-
susception throughout the bowel and
not just in the ileocolic area.1#{ 176} I ntus-
susception appears as a rounded mass
with echocentric structures described
as a sliced onion appearance. I n
addition, ultrasonography can reveal
intestinal wall thickening and abnor-
mal peristalsis. These findings are
highly suggestive of HSP.
Pancreatitis may occur with acute
onset of vomiting and elevated serum
1 1 Hydrops of the gallblad-
der has been described, with right
upper quadrant pain and mass. 2
M assive gastric hemorrhage can oc-
cur secondary to stress ulceration or
use of Finally, ileai
stricture has been reported to occur
months after resolution of the acute
illness.
GENI TOURI NARY TRACT
The long-term outcome of patients
who have HSP is determined by the
extent of renal involvement. The ex-
act prevalence of renal involvement
is unknown, but varies between 20%
and 50% , the frequency varying in
part with the adequacy of examina-
tion. Persistent nephropathy occurs in
about 1% of all cases and progres-
sion to end-stage renal disease in
Patients who develop renal in-
volvement generally do so within 3
mo of the onset of the rash. I n 3% of
cases, renal involvement may pre-
cede the purpura by months. Gas-
trointestinal and renal involvement
usually occur together, and persist-
ence of the rash for 2 to 3 mo is as-
sociated with nephropathy. Episodic
purpura can be associated with recru-
descence of renal disease. I n general,
there is no consistent relationship be-
tween the severity of nephritis and
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HEMATOLOGY
i-Schoenl#{149}ln Purpura
a
FIGURE 5. Unifonn regular thickening of ileal folds simulating
a stack of coins on barium small bowel series (arrows) in an
individual who has Henoch-Schoenlein purpura. Thick bowel
wall due to intramural hemorrhage (h) is responsible for the
separation of bowel loops from each other.
4. h, hemorrhage in wall; i, coil-spring appearance of
transient small bowel intussusception. Parallel symmetric spike-
like configuration (arrows) of ileal folds on barium small bowel
series in an individual who has Henoch-Schoenlein purpura.
Separation of affected from adjacent loops is indicative of
transmural edema and hemorrhage.
FIGURE 6. Thumbprinting (arrows) in the duodenum of a
patient who has Henoch-Schoenlein purpura on barium series.
Pediatrics in Review VoL 13 No. 4 April 1992
133
L
the severi ty of extrarenal mani f esta-
ti ons.
Cl i ni cal expressi on of renal di sease
i n associ ati on wi th HSP ranges f rom
transi ent i sol ated mi croscopi c hema-
tuna to rapi dl y progressi ve gl omeru-
l onephri ti s. The most common
mani f estati on i s hematuri a, whi ch i s
detected i n nearl y al l cases of renal
di sease. There i s a l ower i nci dence
of hematuri a pl us protei nuri a. A f -
f ected pati ents may devel op a nephni -
ti s or a nephri ti c-nephroti c syndrome.
Nephri ti c syndrome i s def i ned as he-
maturi a i n combi nati on wi th hyper-
tensi on, azotemi a, and ol i guri a.
Nephroti c syndrome i s def i ned by
l aboratory resul ts that f i nd a 24-hour
uri ne protei n excreti on >50 mgf kg
per day and a serum al bumi n l evel of
<2.5 mg/dL . 6
Chi l dren who have hematuri a
al one do not devel op chroni c end-
stage renal di sease; on the other
hand, 15% of pati ents who have both
hematuri a and protei nuri a devel op
renal f ai l ure. Pati ents af f l i cted wi th
both nephri ti c and nephroti c syn-
drome devel op end-stage renal di s-
ease i n 50% of cases wi thi n 10 y.
Persi stence of nephroti c-range pro-
tei nuri a has been shown to be the
most accurate predi ctor of eventual
renal f ai l ure, as i n other gl omerul o-
nephropathi es. Chi l dren who are no
l onger nephri ti c or nephroti c and i n
whom resul ts of uri nal ysi s and bl ood
chemi stry val ues f ol l owi ng onset re-
turn to normal do not devel op end-
stage renal di sease.
I n general , the pri mary care physi -
ci an shoul d ref er any chi l d who has
HSP and a nephri ti c or nephroti c
syndrome to a nephrol ogi st; i n such
cases, a renal bi opsy shoul d be
consi dered. 1 6
Renal hi stopathol ogy ranges f rom
mi ni mal change to severe crescenti c
gl omerul onephni ti s. Hypercel l ul ari ty,
segmental scl erosi s, f i brosi s, and i n-
f i l trati on of mononucl ear cel l s al l are
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Ta b le 2 . Re la tio n s h Ip o f P e rc e n ta g e o f Glo m e ru la r
Cre s c e n ts to Ris k o f Re n a l Fa ilu re
P ERCENTAGE OF
GLOMERUU HAVI NG
CRES CENTS
FREQUENCY OF RENAL
FAILURE (%)
< 50 4
50-75 25
> 75 67
100 100
Ta b le 3 . ClIn ic a l Ma n ife s ta tio n s o f Ce n tra l Ne rv o u s
S y s te m
CENTRAL
In v o lv e m e n t in He n o c h -S c h o e n le in P u rp u ra
P ERIP HERAL NERVOUS S YS TEM NERVOUS S YS TEM
r
F I GUR E 7. E ,yth em a an d edem a of
scrotal wall in an individua l who ha s
H en och -S ch oen lein pwpur a .
134 P edia tr ics in Review V oL 13 N o. 4 A pril 1992
H E M A TO LO G Y
H enoch-S chosnl& n P urpura
found on light microscopy . On dcc-
tron microscopy , mesangial, suben-
dothelial, and subepithelial deposits
hav e been seen in that order of fre-
quency . Immunofluorescent studies
rev eal diffuse glomerular deposits
containing IgA , C3, fibnin, IgG, pro-
perdin, and 1gM .3
Renal histology is a useful but not
entirely reliable predictor of renal
outcome. The risk of renal failure in
relationship to the percentage of
glomerular crescents on renal biopsy
is show n in Table 2.
In addition to the renal manifesta-
tions, extranenal genitouninary in-
v olv ement may occur in HSP and
may produce the initial sy mptom. A
v asculitis of scrotal v essels may re-
sult in inflammation and hemorrhage
of the testes, appendix testes, sper-
matic cord, epididy mis, or scrotal
w all. A cute scrotal sw elling occurs in
2% to 35% of cases. HSP may in-
v olv e the scrotal w all, producing v ar-
ious degrees of ery thema and edema
(Fig 7), and may inv olv e the testis
and epididy mis, producing acute
scrotal sw elling mimicking testicular
torsion. A ssociation w ith true torsion
is rare. In boy s w ho hav e sy stemic
manifestations of HSP and acute
scrotal sw elling, technetium 99m
radionuclide scan and ultrasonogra-
phy should be used. Ultrasonographic
findings of acute scrotal hemorrhage
consist of thickening of the scrotal
w all in a hy perechoic pattern rep-
resenting hematoma in the scrotal
w all. Cases can be managed expect-
antly except w hen inv estigation of
acute scrotal sw elling and pain can-
not exclude torsion; then, prompt
surgical exploration is
Other genitourinary complications
include hy dronephrosis, calcified ure-
ten, bladder w all hematoma, urethri-
tis, and hemorrhagic spermatic cord.
H EM A T OL OGI C
In addition to the v asculitis, HSP
also may be complicated by a hemor-
rhagic diathesis. Thrombocy tosis oc-
curs in 67% of cases associated w ith
abdominal pain and gastrointestinal
bleeding. This does not correlate
w ith other clinical features, such as
arthritis or nephritis. Thrombocy tosis
is unexplained, but may represent an
A ltered consciousness
Behav ioral changes
Conv ulsions
Complex partial
Generaliz ed
Partial
Status epilepticus
Headache
Focal deficits
A phasia
A taxia
Chorea
Cortical blindness
Hemiparesis
Paraparesis
Quadriplegia
expression of acute inflammation and
usually is correlated w ith an elev ated
ery throcy te sedimentation
Other hematologic complications in-
dude Factor V III deficiency , v itamin
K deficiency , and hy poprothrombine-
19 These abnormalities lead to a
coagulopathy , w hich can complicate
the course of the illness further. Pul-
monary hemorrhage w ith hemopty sis
has been described, as has hemor-
rhage into large muscle groups,
w hich may produce excruciating
pain.202
CENTRA L NERV OUS SY STEM
The central nerv ous sy stem may suf-
fer from the combined effects of v as-
culitis and hemorrhagic diathesis.
In 1914, Osler reported a child w ho
had allergic purpura associated w ith
hemiplegia. Central nerv ous sy stem
inv olv ement is distinctly uncommon;
w hen it occurs, the most common
sy mptom is headache (Table 3). Sub-
tie changes in mental status may oc-
cur, w ith lability of mood, irrita-
bility , apathy , and hy peractiv ity .
Transient electroencephalographic ab-
normalities hav e been noted. Seiz ures
rarely occur and may precede other
sy stemic manifestations. Hy perten-
sion secondary to renal inv olv ement,
electroly te dy sfunction secondary to
gastrointestinal disease, and central
nerv ous sy stem hemorrhage may be
predisposing factors. Subdural hema-
toma, cortical hemorrhage, intrapar-
enchy mal bleeding, and infarction all
M ononeuropathies
Facial nerv e
Femoral nerv e
Peroneal nerv e
Poly radiculoneuropathies
Brachial plexus neuropathy
Guillian-Barr#{ 233} sy ndrome
Sciatic nerv e
Ulnar nerv e
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r - HEMATOLOGY
Hs no c h-S c ho e nl&nP urpura
[ Table 4. DIfferential Diagnosis of Henoch-Schoenleln
F P urpura and Appro pria te La bo ra to ry Te s ts
DIFFERENTIAL DIAGNOSIS LABORATORY TES TS
Abdomina l P a in
A cute abdominal pain A bdominal roentgenogram
Ultrasonography
Intussusception Barium enema
Ultrasonography
J oint P a ins
A cute rheumatic fev er A ntistreptoly sin 0 titre
Cardiac echocardiography
Chest roentgenogram
Electrocardiogram
Poly arteritis nodosa A ngiography (celiac, inferior
mesenteric, renal)
Histology (kidney s, muscle, skin,
testes)
Rheumatoid arthritis A ntinuclear antibody
CH5O
Quantitativ e immunoglobulins
Rheumatoid factor
Sy stemic lupus ery thematosus A ntinuclear antibody
C3, C4, and CH5O
Ra sh
Child abuse Computed tomography of head
Skeletal surv ey
Drugs (eg, iodide, thiaz ide, mercury ) Drug screening
Hemorrhagic diathesis (eg, idiopathic Coagulation profile
thrombocy topenic purpura, clotting Platelet count
factor deficiency )
Septicemia (eg, meningococcemia) Bacterial culture
Rena l Disea se
A cute glomerulonephritis Blood urea nitrogen
Creatinine
Urinaly sis
Pe d i a t r i c s i n Review V oL 13 No . 4 A pril 1992 135
hav e been reported. Focal neurologic
deficits occur, reflecting inflamma-
tory , ischemic, or hemorrhagic insult
to the central nerv ous sy stem.
Rarely , peripheral neuropathies hav e
been described. Intracranial compli-
cations may be manifested by sudden
changes in behav ior or lev el of
consciousness.
Differential Diagnosis
The full-blow n picture of HSP-fea-
turing ty pical rash, arthritis, and gas-
trointestinal and renal manifes-
tations-is diagnostic. Diagnostic dif-
ficulties arise w hen one sy mptom
predominates or multiple sy stem in-
v olv ement is not recogniz ed. The
elements of the differential diagnosis
of HSP and the appropriate labora-
tory tests are listed in Table 4.
La bo ra to ry Findings
Diagnosis of HSP depends primarily
on clinical findings and history . Lab-
oratory tests are helpful but nondi-
agnostic (Table 5). A complete blood
count may rev eal a normal or dc-
v ated w hite blood cell count and
eosinophilia. A nemia may occur 5cc-
ondary to acute bleeding. Ery throcy te
sedimentation rate and platelet count
may be elev ated in association w ith
acute inflammation. Electroly tes may
be affected secondary to gastrointes-
tinal inv olv ement. Serum amy lase
w ould be elev ated in association w ith
pancreatitis. The hemorrhagic dia-
thesis of HSP is secondary to de-
creased factor V I!! lev els that may
not be reflected in routine prothrom-
bin and partial thromboplastin times.
Routine urinaly sis may show he-
maturia, but any ev idence of protein-
uria should prompt measurement of
24-h urine protein excretion or mea-
surement of the ratio of urine protein
to creatinine, and a serum albumin
lev el should be determined. Because
renal manifestations may follow the
onset of the illness by up to 3 mo,
urinaly sis should be performed
monthly for 3 mo to exclude this late
complication. Blood urea nitrogen
and creatinine lev els w ill be elev ated
w hen there is associated renal in-
v olv ement w ith renal insufficiency .
Renal biopsy should be carried out
w here indicated.
Tests for stool guaiac must be
Testicula r Swelling a nd P a in
Incarcerated hernia
Orchitis
Torsion of testis
done and are positiv e for occult
blood in 49% of cases.
In addition to these routine labora-
tory studies, the choice of diagnostic
tests w ill depend upon the clinical
course of the illness. A ny suggestion
of hy drops of the gallbladder should
be follow ed by liv er function tests
and ultrasonography . A bdominal
roentgenograms, barium enema, and
ultrasonography should be used to
explore the possibility of intraabdom-
inal emergencies. Computed tomog-
raphy of the head should be carried
Surgical exploration
Technetium 99m radionuclide scan
Ultrasonography
out if any neurologic sy mptoms or
signs are present. Chest roentgenog-
raphy should follow any episode of
hemopty sis.
Tests more specific for HSP in-
dude determining the lev el of serum
IgA , w hich may be elev ated or nor-
mal. Skin biopsy of purpural lesions
show s leukocy toclastic v asculitis w ith
IgA deposits. A n underly ing infec-
tious etiologic agent (eg, streptococ-
cal infection or infectious mono-
nucleosis) should be excluded w here
clinically indicated.
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HEMATOLOGY
H#{149}noch-Schoeni#{149}lnPurpura
Ta b le 5 . La b o r a t o r y Te s t s a n d E x p e c t e d F in d in g s In
Henoch-Schoenleln P u r p u r a
LAB O R ATO R Y TE S TS E XP E C TE D F INDING S
Blood Chemist,y
A lbum in Hy poalbum inem ia, occasionally
A m y lase lev el R aised in association w ith pancreatitis
B lood urea nitrogen lev el R aised in association w ith renal
disease
Electroly tes Generally norm al
S erum creatinine lev el R aised in association w ith renal
disease
Coagulation Factors
Prothrom bin tim e and partial N orm al
throm boplastin tim e
Factor V III Decreased
Factor X III Decreased
Hematology
Ery throcy te sedim entation rate M ildly elev ated in 75% of cases
Hem oglobin N orm al or low secondary to bleeding
Platelet count N orm al or elev ated
W hite cell count L euk ocy tosis w ith lef t shif t
Immunologic Tests
A ntinuclear antibody N egativ e
A ntistreptoly sin 0 titre E levated in 30% of cases
C3 and C4 lev els Occasionally low
CH5O lev els L ow in 30% of cases
Circulating IgA and IgG im m une Increased
com plex es lev els
IgG rheum atoid factor N egative
M onocy tic and m acrophage Decreased
Fc-receptor f unction
S erum IgA lev el Elev ated in 50% of cases
S erum im m unoglobulin lev el Increased early during the course of
disease
Radiologic studies
A bdom inal roentgenogram Free air-perf oration
Fluid lev els and intussusception
A bdom inal ultrasonography Intussusception
B arium enem a B lurring of sharpness of f olds
Coil-spring appearance
Filling def ects
S eparation of bow el loops
S pik e-lik e appearance
S tack of coins appearance
T hum bprinting
Renal Biopsy
R enal biopsy Dif f use prolif eration w ith or w ithout
crescents
Focal and segm ental prolif eration
IgA deposits in m esangium
M esangial prolif eration
N orm al
Skin Biopsy
S k in biopsy L euk ocy toclastic v asculitis
Stool
S tool guaiac Positiv e in 50% of cases
Urinas ysis
B lood, casts, protein
136 Pediatrics in Review VoL 13 No. 4 April 1992
Present in association w ith renal
disease
Tr e a t m e n t
N o specif ic treatm ent is av ailable f or
HS P, and treatm ent is supportiv e. In
acute cases, adequate hy dration
should be prov ided and the patient
should be m onitored f or com plica-
tions. Frequent assessm ent of v ital
signs, hem atocrit, stool ex am ination
f or blood, and abdom inal ex am ina-
tions should be conducted. A ll un-
necessary drugs should be discon-
tinued, allergen ex posure av oided,
and underly ing illnesses and com pli-
cations treated.
N onsteroidal antiinf lam m atory
agents reliev e joint and sof t tissue
discom f ort. Corticosteroids hav e a f a-
v orable ef f ect on sof t tissue sw elling,
and on joint, scrotal, and gastrointes-
f inal disease. In instances of abdom i-
nal colic and gastrointestinal hem or-
rhage, im pressiv e relief occurs w ith
prednisone, giv en in a dose of 1 to 2
m g/k g per day f or 5 to 7 day s. T he
response is of ten rapid and strik ing.
Corticosteroids are not recom -
m ended, how ev er, f or rash, edem a,
joint pains, or renal disease alone.
M ajor m anif estations of localiz ed
v asculitis in the lungs, testes, and
central nerv ous sy stem should be
treated w ith corticosteroids.
T he m anagem ent of nephropathy
requires attention to f luid balance,
electroly te status, salt intak e, and use
of antihy pertensiv e agents w hen m di-
cated. V arious com binations of im -
m unosuppressiv e drugs, including
corticosteroids, hav e been used to
treat renal disease; how ev er, the use-
f ulness of this approach has not been
clearly established by controlled
study . Plasm apheresis has been re-
ported to reliev e som e renal decom -
pensation.3 A recent study reported
that prednisone giv en early in a dose
of 1 to 2.5 m g/k g per day f or 21
day s m ay reduce the incidence of ne-
phropathy , but m ost current litera-
ture suggests that im m unosuppressiv e
drugs are contraindicated in the treat-
m ent of HS P nephritis. T his recent
recom m endation requires f urther
study bef ore its use is adopted. Corti-
costeroids hav e been reported to be
benef icial in the treatm ent of both in-
trapulm onary hem orrhage and renal
f ailure.24
A study from Japan found that de-
creased f actor V III lev els w ere corre-
lated w ith m ore sev ere clinical
sy m ptom s, particularly w ith respect
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PI R QUI Z
1. Syn on ym s for H en och -Sch oen lein
purpura (H SP ) h a ve in clu d ed ea ch
of the follow ing except:
A. L eu k ocyt ocla st ic p u r p u r a .
B. A naphy lactoid purpura.
C. Berger disease.
D. Aller gic va scu lit is.
E. Rheumatoid purpura.
2. A mong the follow ing, the least
likely laboratory abnormality
associated w ith HSP is:
A. T h r om b ocyt op en ia .
B. E leva t ed IgA lev el in plasma.
C. Decreased factor V III lev el in
blood.
D. P r ot ein u r ia .
E . E leva t ed er yt h r ocyt e
sedimentation rate.
3. The histologic pattern of the renal
lesion in association w ith HSP is
identical w ith that found in:
A. Acu t e glom er u lon ep h r it is.
B. IgA nephropathy .
C . Syst em ic lu p u s er yt h em a t osu s.
D. L ip oid n ep h r osis.
E . Segm en t a l n ep h r it is.
4. E a ch of t h e followin g is a t r u e
statement regarding HSP, except:
A. T h e com p lica t ion of
intussusception in HSP is
more oft en ileoilea l t h a n
ileocolic.
B. Ult r a son ogr a p h y is t h e m et h od
of choice in eva lu a t ion of t h e
child w ho has HSP and w ho
has abdominal pain.
C. A hemorrhagic diathesis may
b e a ssocia t ed wit h sever e
abdominal pain in children
wh o h a ve H SP .
D. R ecu r r en t a r t h r it is in H SP
commonly leads to chronic
joint disability .
E . T h e lon g-t er m p r ogn osis in
H SP is m ost closely r ela t ed t o
the sev erity of the renal
lesion.
Pediatrics in R eview V oL 13 N o. 4 A pril 1 9 9 2
137
H E M A TO LO G Y
J Hnoch-Schosnlsln P urpura
to abdominal sy mptoms. In a con-
trolled study , sy mptoms w ere re-
markably improv ed follow ing 3 day s
of administration of factor V III. Fac-
tor V III lev els became normal and
sy mptoms resolv ed. The usefulness
of the administration of factor V III in
the treatment of HSP requires further
study .
Prognosis
In the absence of renal disease and
major central nerv ous sy stem in-
v olv ement, the prognosis for indiv id-
uals w ho hav e HSP is excellent. The
illness lasts 4 to 6 w k in the majority
of cases. Recurrences, w hich occur
in half the patients, usually occur
w ithin 6 w k, but may be noted as
late as 7 y after the onset of illness.
Children y ounger than 3 y tend to
hav e a shorter, milder course and
few er recurrences. In rare cases,
long-term morbidity occurs because
of residual central nerv ous sy stem
damage.
Long-term follow -up is necessary
in patients w ho hav e renal inv olv e-
ment because progression of renal
disease may not occur for many
y ears. M icroscopic hematuria alone
is associated w ith good long-term
prognosis, but more sev ere renal in-
v olv ement has a v ariable outcome. A
poor prognosis w ith progression to
renal failure has been associated w ith
the onset of HSP nephritis in children
older than 6 y , the dev elopment of
the nephrotic sy ndrome, and the
presence of crescent formation in
more than 50% of the glomeruli. A
renal biopsy can help to establish the
diagnosis and determine prognosis.
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W e wou ld lik e t o t h a n k Drs Sam W einberg
a n d W illia m Br ock for t h e p h ot ogr a p h s of
t h e sk in lesion s a n d Dr J oh n L eon id a s for
t h e r a d iogr a p h s.
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DOI:10.1542/pir.13-4-130
1992;13;130-137 Pediatr. Rev.
Shelley Lanzkowsky, Leora Lanzkowsky and Philip Lanzkowsky
Henoch-Schoenlein Purpura

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