henoch schoenlein purpura

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 PurpuraShelley Lanzkowsky, MD,* Leora Lanzkowsky, MD,** andPhilip Lanzkowsky, MD***

FOCUS QUESTIONS

1. What is our understanding of thepathophysiology of Henoch.

Schoenlein purpura (HSP)?

2. What are the cardinal clinical fea-

tures of HSP?

3. Which clinical features offer sub-stantial threat to survival or full

recovery from HSP?4. What clinical or laboratory obser-

vations are required for or appro-

pilate to monitoring the course of

HSP?

5. What therapeutic measures areavailable for HSP, and what clini-

cal features direct their choice anduse?

Altending Pediatrician, Children’s Special-

ized Hospital, Mountainside, New Jersey.

5�Dep��tmerJ of Radiology, Long Island

Jewish Medical Center, New Hyde Park, New

York.

� Department of Pediatrics;

Chief-of-Staff, Schneider Children’s Hospital,Long Island Jewish Medical Center; Profes-

sor of Pediatrics, Albert Einstein College ofMedicine. Address reprint requests to: P.Lanzkowsky, Department of Pediatrics,

Schneider Children’s Hospital, Long IslandJewish Medical 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 firstdescribed by Heberden before 1800,and in 1808 English physician RobertWillan described a patient who hadswollen, painful joints and a rash.

The syndrome owes its name totwo German physicians. In 1837,Johan Schoenlein described severalcases of purpura associated with ar-thritis, which he termed “peliosisrheumatica.” In 1868, EdouardHenoch pointed out that the term“peliosis rheumatica” was inappro-priate and restrictive because patientswho had urticarial purpura and acutearthritis also manifested gastrointes-tinal symptoms, such as vomiting,colicky abdominal pain, and melena.He described four children who hadpurpura, colicky abdominal pain, gastro-intestinal hemorrhage, and joint painas well as renal involvement) Sincethese first descriptions, HSP also hasbeen referred to as anaphylactoid, al-

lergic, or rheumatoid purpura; leuko-cytoclastic vasculitis; and allergic

1.2

EpidemiologySeventy-five percent of all cases ofHSP occur in children between 2 and1 1 years of age, with younger chil-dren rarely affected. Childrenyounger than 2 y experience a mildercourse of illness, with less frequentrenal and gastrointestinal manifesta-tions.3 HSP is exceedingly rare inadults, among whom other causes ofvasculitis should be considered morelikely.

The incidence of HSP is greater inmales, with a male-to-female ratio of1.5 to 2.0:1. In adults, the sex ratioapproaches 1 : 1. There appears to bea seasonal incidence, with morecases in the spring and autumnmonths. Recent reports indicate thatHSP may be less common in blackthan white children.4

EtiologyThe etiology of HSP-like that of asimilar vasculitis, Kawasaki dis-ease-is unresolved. In 1914, Wil-ham Osler hypothesized an allergicbasis for the illness.5 Seventy-fivepercent of patients give a history ofupper respiratory tract infection pre-ceding the onset of this syndrome.Streptococcal pharyngitis once wasthought to play a large role in patho-genesis, but studies have shown thatthe incidence of elevated streptococ-cal antibody titer in patients whohave HSP is no higher than that ofcontrols matched for age.3

Other infectious agents reported tohave preceded HSP have includedthose due to Yersinia, Legionella,

parvovirus, adenovirus, Mycoplasma,Epstein-Barr virus, and varicella.5’6Cases 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 etiologyhas been established; seasonal varia-tion in incidence has been noted, butno clear-cut pattern is apparent.

PathogenesisHSP is thought to be an IgA-me-diated vasculitis in small vessels ofinvolved organs. IgA levels becomeelevated either because of increasedproduction or decreased renal clear-ance of IgA. It is postulated that anunknown antigenic stimulant causeselevated IgA, activating pathwaysleading to necrotizing vasculitis.Lymphocytes, neutrophils, circulatingimmune complexes, IgA, C3, and fi-brin infiltrate systemic blood

Despite differences in clinical pre-sentation, the renal lesion of HSP isindistinguishable histopathologicallyfrom that of IgA nephropathy (Bergerdisease). Renal involvement occursin 20% to 50% of patients who haveHSP; approximately 30% of patientswho have Berger disease have extra-renal manifestations of rash and ar-thralgia. Both diseases result inelevated IgA production, increasedcirculating immune complexes ofIgA, 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, ingeneral, younger than those whohave IgA nephropathy, but the agedistribution overlaps. Both diseasescarry the risk of progression to renalinsufficiency, and recurrent diseasedevelops in both following renaltransplantation. The similarity of fea-tures in these two illnesses can beexplained by a similar pathogenesiswith different expressions in thehosts. In the immature host, the cir-culating immune complexes maygenerate systemic HSP, whereas theolder host may develop only renalmanifestations of the illness.4

Clinical ManifestationsThe hallmarks of HSP are a non-thrombocytopenic purpuric rash, ab-dominal pain, arthritis, and nephritis.The illness may follow various clini-cal courses, however. The character-istic symptoms may appear in any



Tabl#{149}1. PresentIng Symptoms and Signs InSchonleln Purpura � � �

H.noch-1� � � JI___J

ORGAN SYMPTOMS AND SIGNS

Cardiac Myocardial infarction (coronary vasculitis)

Central nervous system ApathyHeadacheHyperactivityIrritabilityRare symptoms and signs (see Table 2)Somnolence

Gastrointestinal tract Acute appendicitisColicky abdominal painGastrointestinal hemorrhageGuaiac-positive stoolsHematemesisHepatomegalyHydrops of gallbladderIleusIntussusception (ileoileal in 65% of cases)MelenaPancreatitisVomiting

Genitourinary tract Hematuria and proteinuriaMicroscopic hematuriaNephritic and nephrotic signsNephritic signs

Scrotal involvement

Musculoskeletal Arthritis/arthralgiaIntramuscular hemorrhage

Non-specific FeverPreceding infection

Pulmonary System Pulmonary hemorrhageSerosanguinous pleural effusion

Skin Erythematous maculopapulesPetechiaePurpuraSubcutaneous edema (scalp, extremities)Urticaria

Pediatrics in Review VoL 13 No. 4 April 1992 ‘3’

1�-�-� �

H� �

nochHEMATOLOGY I-Schosnl&n Purpura I

. � �-- - �-�

order; accordingly, there may be avariety of clinical presentations. Thepresenting symptoms and signs in

HSP are listed in Table 1.

SKIN

The typical rash occurs in 100% ofcases and is the presenting feature in50% of cases. Lesions appear on thelower extremities and buttocks, butmay involve upper extremities, face,and trunk. The classic lesions consistof urticarial wheals, erythematousmaculopapules similar to those foundin erythema multiforme, or largerpalpable ecchymotic-looking lesions(Figs 1-3). In addition to these char-acteristic lesions, various patterns oferythema multiforme may occur. Le-sions initially blanch on pressure, butlater lose this feature. Petechiae orpurpuric lesions occur, but there isno associated thrombocytopenia. Thepurpuric areas evolve from red topurple, becoming rust-colored with abrownish hue, and eventually fade.New crops may arise, giving a poly-morphic appearance. Occasionallythe 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 haveerythema multiforme with centralhemorrhage or ulceration and forma-

tion of bullae.7 Furthermore, the typ-ical distribution over the buttocksmay be completely absent, and HSPhas been described with orofacial le-sions alone.

Angioedema of the scalp and ofthe extremities occurs in 20% and46% of cases, respectively. Angio-edema is nonpitting; may involveeyelids, lips, dorsa of the hands andfeet, back, and perineum; and maybe especially striking in childrenyounger than 3 y. Rarely, an entirelimb may be transiently swollen andtender.

JOINTS

Arthralgia, arthritis, or both occur in68% to 75% of cases. They precedethe rash and are the first signs ofHSP in approximately 25% of pa-tients.3 Joint involvement tends to beperiarticular, usually without bleed-ing or effusion into joints. Affectedjoints may be swollen, tender, andpainful on motion. The tenderness

and swelling are mainly periarticular.Ankles and knees are the most com-monly affected joints, whereas thefingers and wrists usually are not in-volved. Joint involvement is transientor may recur during active disease,but leaves no permanent deformity.

GASTROINTESTINAL TRACT

The most frequent symptom of HSPfollowing rash and joint pain is ab-dominal pain, which occurs in ap-proximately 35% to 85% of cases. Inthe majority of cases, cutaneousmanifestations of HSP precede ab-dominal symptoms, but visceral man-ifestations precede the rash in 14% of

cases. The most common complaintis colicky abdominal pain, whichmay be severe and associated withvomiting. Stools show gross or oc-cult blood in more than half of thecases, and hematemesis may occur.Abdominal pain associated with HSPis occasionally difficult to distinguishfrom other causes of abdominal painand may mimic that of an intraab-dominal emergency.

Most cases of abdominal pain aredue to submucosal and intramural cx-travasation of fluid and blood intothe intestinal wall, which may lead tolocalized ulceration of the mucosa(causing guaiac-positive stools) and



HEMATOLOGYH#{149}noch-Schoinleln Purpura

FIGURE!. Fairly well defined

e�ythematous, urticarial lesions with

some slight petechial lesions on posterior

aspect of lower extremity of an individual

who has Henoch-Schoenlein purpura.

Typical location for these lesions.

FIGURE 2. Fairly well definedetythematous, urticarial lesions with

some slight petechial lesions on lower leg

and ankle of an individual who has

Henoch-Schoenlein puipura. Typical

location for these lesions.

FIGURE 3. An e,ythematous, urticaria4

slightly edematous lesion that is illdefined on helix and antihelix of ear of

an individual who has Henoch-

Schoenlein purpura. Not a common

location for this condition.


may be associated with diffuse arte-rial inflammation and fibrinoid necro-sis. This is due to mesentericvasculitis.

The radiographic manifestations ofHSP are nonspecific, and othercauses of intramural bleeding (var-ious coagulopathies or other forms ofvasculitis) and inflammatory changes(such as inflammatory bowel disease)may appear similar. Clinical correla-tion is extremely important.

The following six gastrointestinalradiographic findings are consistentwith a diagnosis of HSP8: 1) uniformregular thickening of small bowelwall folds, causing parallel, symme-tric, spike-like configurations simu-lating a stack of coins or picketfence, most striking in the jejunum(Figs 4 and 5); 2) blurring of thesharpness and symmetry of the smallbowel wall folds, due to edema ofthe small bowel and increased secre-tions; 3) thumbprinting and scallop-ing due to local hemorrhage of bowelwall (Fig 6); 4) filling defects inbowel wall due to mucosal edemaand small areas of dilatation as a re-suit of vascular occlusion in the sub-mucosa; 5) intramural or extramuralmasses, flattening of the folds on themesenteric side, and separation anduncoupling of the bowel loops, rep-resenting bleeding into the mesentry;6) coil-spring appearance of smallbowel intussusception (Fig 4). Thesenonspecific radiographic findings allare transient and usually resolve

within 4 to 6 wk without any residualdefects.

A sudden onset of or increase inabdominal pain associated with HSPmay be secondary to intussusception,bowel infarction, bowel perforation,pancreatitis, or hydrops of the gallbladder.

Intussusception occurs in 2% to3% of patients, but infarction andperforation of the bowel are uncom-mon. The incidence of abdominalconditions requiring surgery in asso-ciation with HSP is 2% to 6%. Mostsurgical complications occur after theappearance of purpura. Although ab-dominal complaints that appear priorto the rash may be severe, they areunlikely to be due to infarction ne-crosis or perforation of the gut.9

Intussusception associated withHSP is often the result of a submu-cosal hematoma and is ileoileai in65% of cases, whereas 95% of casesof intussusception in the populationat large are ileocolic. A bariumenema 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 andnot just in the ileocolic area.1#{176}Intus-susception appears as a rounded masswith echocentric structures describedas a “sliced onion” appearance. Inaddition, ultrasonography can revealintestinal wall thickening and abnor-mal peristalsis. These findings are

highly suggestive of HSP.Pancreatitis may occur with acute

onset of vomiting and elevated serum1 1 Hydrops of the gallblad-

der has been described, with rightupper quadrant pain and mass.’2Massive gastric hemorrhage can oc-cur secondary to stress ulceration oruse of � Finally, ileaistricture has been reported to occurmonths after resolution of the acuteillness. ‘�

GENITOURINARY TRACT

The long-term outcome of patientswho have HSP is determined by theextent of renal involvement. The ex-act prevalence of renal involvementis unknown, but varies between 20%and 50%, the frequency varying inpart with the adequacy of examina-tion. Persistent nephropathy occurs inabout 1% of all cases and progres-sion to end-stage renal disease in

Patients who develop renal in-volvement generally do so within 3mo of the onset of the rash. In 3% ofcases, renal involvement may pre-cede the purpura by months. Gas-trointestinal and renal involvementusually occur together, and persist-ence of the rash for 2 to 3 mo is as-sociated with nephropathy. Episodicpurpura can be associated with recru-descence of renal disease. In general,there is no consistent relationship be-tween the severity of nephritis and



HEMATOLOGYi-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 anindividual who has Henoch-Schoenlein purpura. Thick bowelwall 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 oftransmural 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 severity of extrarenal manifesta-tions.

Clinical expression of renal diseasein association with HSP ranges fromtransient isolated microscopic hema-tuna to rapidly progressive glomeru-lonephritis. The most commonmanifestation is hematuria, which isdetected in nearly all cases of renaldisease. There is a lower incidenceof hematuria plus proteinuria. Af-fected patients may develop a nephni-tis or a nephritic-nephrotic syndrome.Nephritic syndrome is defined as he-maturia in combination with hyper-tension, azotemia, and oliguria.Nephrotic syndrome is defined bylaboratory results that find a 24-hoururine protein excretion >50 mgfkgper day and a serum albumin level of<2.5 mg/dL.’6

Children who have hematuriaalone do not develop chronic end-

stage renal disease; on the otherhand, 15% of patients who have bothhematuria and proteinuria developrenal failure. Patients afflicted withboth nephritic and nephrotic syn-drome develop end-stage renal dis-ease in 50% of cases within 10 y.Persistence of nephrotic-range pro-teinuria has been shown to be themost accurate predictor of eventualrenal failure, as in other glomerulo-nephropathies. Children who are nolonger nephritic or nephrotic and inwhom results of urinalysis and blood

chemistry values following onset re-turn to normal do not develop end-stage renal disease.

In general, the primary care physi-cian should refer any child who hasHSP and a nephritic or nephroticsyndrome to a nephrologist; in suchcases, a renal biopsy should beconsidered. 16

Renal histopathology ranges fromminimal change to severe crescenticglomerulonephnitis. Hypercellularity,segmental sclerosis, fibrosis, and in-filtration of mononuclear cells all are



Table 2. RelationshIp of Percentage of GlomerularCrescents to Risk of Renal Failure

PERCENTAGE OFGLOMERUU HAVING

CRESCENTSFREQUENCY OF RENAL

FAILURE (%)

<50 4

50-75 25

>75 67

100 100

Table 3. ClInical Manifestations of Central NervousSystem

CENTRAL

Involvement in Henoch-Schoenlein Purpura

PERIPHERAL NERVOUS SYSTEMNERVOUS SYSTEM

r

FIGURE 7. E,ythema and edema ofscrotal wall in an individual who hasHenoch-Schoenlein pwpura.


HEMATOLOGYHenoch-Schosnl&n Purpura

found on light microscopy. On dcc-tron microscopy, mesangial, suben-dothelial, and subepithelial depositshave been seen in that order of fre-quency. Immunofluorescent studiesreveal diffuse glomerular depositscontaining IgA, C3, fibnin, IgG, pro-perdin, and 1gM.3

Renal histology is a useful but notentirely reliable predictor of renaloutcome. The risk of renal failure inrelationship to the percentage ofglomerular crescents on renal biopsyis shown in Table 2.�

In addition to the renal manifesta-tions, extranenal genitouninary in-volvement may occur in HSP andmay produce the initial symptom. Avasculitis of scrotal vessels may re-sult in inflammation and hemorrhageof the testes, appendix testes, sper-matic cord, epididymis, or scrotalwall. Acute scrotal swelling occurs in2% to 35% of cases. HSP may in-volve the scrotal wall, producing var-ious degrees of erythema and edema(Fig 7), and may involve the testisand epididymis, producing acutescrotal swelling mimicking testiculartorsion. Association with true torsionis rare. In boys who have systemicmanifestations of HSP and acutescrotal swelling, technetium 99m

radionuclide scan and ultrasonogra-phy should be used. Ultrasonographicfindings of acute scrotal hemorrhageconsist of thickening of the scrotalwall in a hyperechoic pattern rep-resenting hematoma in the scrotalwall. Cases can be managed expect-antly except when investigation ofacute scrotal swelling and pain can-not exclude ‘torsion; then, promptsurgical exploration is �

Other genitourinary complicationsinclude hydronephrosis, calcified ure-ten, bladder wall hematoma, urethri-tis, and hemorrhagic spermatic cord.

HEMATOLOGIC

In addition to the vasculitis, HSPalso may be complicated by a hemor-rhagic diathesis. Thrombocytosis oc-curs in 67% of cases associated withabdominal pain and gastrointestinalbleeding. This does not correlatewith other clinical features, such asarthritis or nephritis. Thrombocytosisis unexplained, but may represent an

Altered consciousnessBehavioral changesConvulsions

Complex partialGeneralizedPartialStatus epilepticus

HeadacheFocal deficits

AphasiaAtaxiaChoreaCortical blindnessHemiparesisParaparesisQuadriplegia

expression of acute inflammation andusually is correlated with an elevatederythrocyte sedimentation �Other hematologic complications in-dude Factor VIII deficiency, vitaminK deficiency, and hypoprothrombine-

19 These abnormalities lead to acoagulopathy, which can complicatethe course of the illness further. Pul-monary hemorrhage with hemoptysishas been described, as has hemor-rhage into large muscle groups,which may produce excruciatingpain.20’2’

CENTRAL NERVOUS SYSTEM

The central nervous system may suf-fer from the combined effects of vas-culitis and hemorrhagic diathesis.�In 1914, Osler reported a child whohad allergic purpura associated withhemiplegia. Central nervous systeminvolvement is distinctly uncommon;when it occurs, the most commonsymptom is headache (Table 3). Sub-tie changes in mental status may oc-cur, with lability of mood, irrita-bility, apathy, and hyperactivity.Transient electroencephalographic ab-normalities have been noted. Seizuresrarely occur and may precede othersystemic manifestations. Hyperten-sion secondary to renal involvement,electrolyte dysfunction secondary togastrointestinal disease, and centralnervous system hemorrhage may bepredisposing factors. Subdural hema-toma, cortical hemorrhage, intrapar-enchymal bleeding, and infarction all

MononeuropathiesFacial nerveFemoral nervePeroneal nervePolyradiculoneuropathies

Brachial plexus neuropathyGuillian-Barr#{233} syndrome

Sciatic nerveUlnar nerve



r � - HEMATOLOGYHsnoch-Schoenl&nPurpura

[ Table 4. DIfferential Diagnosis of Henoch-SchoenlelnF Purpura and Appropriate Laboratory Tests

DIFFERENTIAL DIAGNOSIS LABORATORY TESTS

Abdominal Pain

Acute abdominal pain Abdominal roentgenogram

Ultrasonography

Intussusception Barium enemaUltrasonography

Joint PainsAcute rheumatic fever Antistreptolysin 0 titre

Cardiac echocardiographyChest roentgenogramElectrocardiogram

Polyarteritis nodosa Angiography (celiac, inferiormesenteric, renal)

Histology (kidneys, muscle, skin,testes)

Rheumatoid arthritis Antinuclear antibodyCH5OQuantitative immunoglobulinsRheumatoid factor

Systemic lupus erythematosus Antinuclear antibodyC3, C4, and CH5O

Rash

Child abuse Computed tomography of headSkeletal survey

Drugs (eg, iodide, thiazide, mercury) Drug screening

Hemorrhagic diathesis (eg, idiopathic Coagulation profilethrombocytopenic purpura, clotting Platelet countfactor deficiency)

Septicemia (eg, meningococcemia) Bacterial culture

Renal DiseaseAcute glomerulonephritis Blood urea nitrogen

CreatinineUrinalysis


have been reported. Focal neurologicdeficits occur, reflecting inflamma-tory, ischemic, or hemorrhagic insultto the central nervous system.Rarely, peripheral neuropathies havebeen described. Intracranial compli-cations may be manifested by suddenchanges in behavior or level ofconsciousness.

Differential DiagnosisThe full-blown picture of HSP-fea-turing typical rash, arthritis, and gas-trointestinal and renal manifes-tations-is diagnostic. Diagnostic dif-ficulties arise when one symptompredominates or multiple system in-volvement is not recognized. Theelements of the differential diagnosisof HSP and the appropriate labora-tory tests are listed in Table 4.

Laboratory FindingsDiagnosis of HSP depends primarilyon clinical findings and history. Lab-oratory tests are helpful but nondi-agnostic (Table 5). A complete bloodcount may reveal a normal or dc-vated white blood cell count andeosinophilia. Anemia may occur 5cc-ondary to acute bleeding. Erythrocytesedimentation rate and platelet countmay be elevated in association withacute inflammation. Electrolytes maybe affected secondary to gastrointes-tinal involvement. Serum amylasewould be elevated in association withpancreatitis. The hemorrhagic dia-thesis of HSP is secondary to de-creased factor VI!! levels that maynot be reflected in routine prothrom-bin and partial thromboplastin times.

Routine urinalysis may show he-maturia, but any evidence of protein-uria should prompt measurement of24-h urine protein excretion or mea-surement of the ratio of urine proteinto creatinine, and a serum albuminlevel should be determined. Becauserenal manifestations may follow theonset of the illness by up to 3 mo,urinalysis should be performedmonthly for 3 mo to exclude this latecomplication. Blood urea nitrogenand creatinine levels will be elevatedwhen there is associated renal in-volvement with renal insufficiency.Renal biopsy should be carried outwhere indicated.

Tests for stool guaiac must be

Testicular Swelling and Pain

Incarcerated hernia

Orchitis

Torsion of testis

done and are positive for occultblood in 49% of cases.

In addition to these routine labora-tory studies, the choice of diagnostictests will depend upon the clinicalcourse of the illness. Any suggestionof hydrops of the gallbladder shouldbe followed by liver function testsand ultrasonography. Abdominalroentgenograms, barium enema, andultrasonography should be used toexplore 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 symptoms orsigns are present. Chest roentgenog-raphy should follow any episode ofhemoptysis.

Tests more specific for HSP in-dude determining the level of serumIgA, which may be elevated or nor-mal. Skin biopsy of purpural lesionsshows leukocytoclastic vasculitis withIgA deposits. An underlying infec-tious etiologic agent (eg, streptococ-cal infection or infectious mono-nucleosis) should be excluded whereclinically indicated.



HEMATOLOGY

H#{149}noch-Schoeni#{149}lnPurpura

Table 5. Laboratory Tests and Expected Findings InHenoch-Schoenleln Purpura

LABORATORY TESTS EXPECTED FINDINGS

Blood Chemist,y

Albumin Hypoalbuminemia, occasionallyAmylase level Raised in association with pancreatitisBlood urea nitrogen level Raised in association with renal

diseaseElectrolytes Generally normalSerum creatinine level Raised in association with renal

disease

Coagulation Factors

Prothrombin time and partial Normalthromboplastin time

Factor VIII DecreasedFactor XIII Decreased

Hematology

Erythrocyte sedimentation rate Mildly elevated in 75% of casesHemoglobin Normal or low secondary to bleedingPlatelet count Normal or elevatedWhite cell count Leukocytosis with left shift

Immunologic TestsAntinuclear antibody NegativeAntistreptolysin 0 titre Elevated in 30% of casesC3 and C4 levels Occasionally lowCH5O levels Low in 30% of casesCirculating IgA and IgG immune Increased

complexes levelsIgG rheumatoid factor NegativeMonocytic and macrophage Decreased

Fc-receptor functionSerum IgA level Elevated in 50% of casesSerum immunoglobulin level Increased early during the course of

disease

Radiologic studiesAbdominal roentgenogram Free air-perforation

Fluid levels and intussusceptionAbdominal ultrasonography IntussusceptionBarium enema Blurring of sharpness of folds

Coil-spring appearanceFilling defectsSeparation of bowel loopsSpike-like appearance“Stack of coins” appearance“Thumbprinting”

Renal Biopsy

Renal biopsy Diffuse proliferation with or withoutcrescents

Focal and segmental proliferationIgA deposits in mesangiumMesangial proliferationNormal

Skin BiopsySkin biopsy Leukocytoclastic vasculitis

StoolStool guaiac Positive in 50% of cases

Urinas�ysisBlood, casts, protein


Present in association with renaldisease

TreatmentNo specific treatment is available forHSP, and treatment is supportive. Inacute cases, adequate hydrationshould be provided and the patientshould be monitored for complica-tions. Frequent assessment of vitalsigns, hematocrit, stool examinationfor blood, and abdominal examina-tions should be conducted. All un-necessary drugs should be discon-tinued, allergen exposure avoided,and underlying illnesses and compli-cations treated.

Nonsteroidal antiinflammatoryagents relieve joint and soft tissuediscomfort. Corticosteroids have a fa-vorable effect on soft tissue swelling,and on joint, scrotal, and gastrointes-final disease. In instances of abdomi-nal colic and gastrointestinal hemor-rhage, impressive relief occurs withprednisone, given in a dose of 1 to 2mg/kg per day for 5 to 7 days. Theresponse is often rapid and striking.Corticosteroids are not recom-mended, however, for rash, edema,joint pains, or renal disease alone.Major manifestations of localizedvasculitis in the lungs, testes, andcentral nervous system should betreated with corticosteroids.

The management of nephropathyrequires attention to fluid balance,electrolyte status, salt intake, and useof antihypertensive agents when mdi-cated. Various combinations of im-munosuppressive drugs, includingcorticosteroids, have been used totreat renal disease; however, the use-fulness of this approach has not beenclearly established by controlledstudy. Plasmapheresis has been re-ported to relieve some renal decom-pensation.3 A recent study reportedthat prednisone given early in a doseof 1 to 2.5 mg/kg per day for 21days may reduce the incidence of ne-phropathy,� but most current litera-ture suggests that immunosuppressivedrugs are contraindicated in the treat-ment of HSP nephritis. This recentrecommendation requires furtherstudy before its use is adopted. Corti-costeroids have been reported to bebeneficial in the treatment of both in-trapulmonary hemorrhage and renalfailure.24

A study from Japan found that de-creased factor VIII levels were corre-lated with more severe clinicalsymptoms, particularly with respect



PIR QUIZ

1. Synonyms for Henoch-Schoenleinpurpura (HSP) have included eachof the following except:

A. Leukocytoclastic purpura.B. Anaphylactoid purpura.C. Berger disease.D. Allergic vasculitis.E. Rheumatoid purpura.

2. Among the following, the leastlikely laboratory abnormalityassociated with HSP is:A. Thrombocytopenia.B. Elevated IgA level in plasma.C. Decreased factor VIII level in

blood.D. Proteinuria.E. Elevated erythrocyte

sedimentation rate.

3. The histologic pattern of the renallesion in association with HSP isidentical with that found in:A. Acute glomerulonephritis.B. IgA nephropathy.C. Systemic lupus erythematosus.D. Lipoid nephrosis.E. Segmental nephritis.

4. Each of the following is a truestatement regarding HSP, except:A. The complication of

intussusception in HSP ismore often ileoileal thanileocolic.

B. Ultrasonography is the methodof choice in evaluation of thechild who has HSP and whohas abdominal pain.

C. A hemorrhagic diathesis maybe associated with severeabdominal pain in childrenwho have HSP.

D. Recurrent arthritis in HSPcommonly leads to chronicjoint disability.

E. The long-term prognosis inHSP is most closely related tothe severity of the renallesion.


HEMATOLOGYJ Hnoch-Schosnlsln Purpura

to abdominal symptoms. In a con-trolled study, symptoms were re-markably improved following 3 daysof administration of factor VIII. Fac-tor VIII levels became normal andsymptoms resolved.� The usefulnessof the administration of factor VIII inthe treatment of HSP requires furtherstudy.

PrognosisIn the absence of renal disease andmajor central nervous system in-volvement, the prognosis for individ-uals who have HSP is excellent. Theillness lasts 4 to 6 wk in the majorityof cases. Recurrences, which occurin half the patients, usually occurwithin 6 wk, but may be noted aslate as 7 y after the onset of illness.Children younger than 3 y tend tohave a shorter, milder course andfewer recurrences. In rare cases,long-term morbidity occurs becauseof residual central nervous systemdamage.

Long-term follow-up is necessaryin patients who have renal involve-ment because progression of renaldisease may not occur for manyyears. Microscopic hematuria aloneis associated with good long-termprognosis, but more severe renal in-volvement has a variable outcome. Apoor prognosis with progression torenal failure has been associated withthe onset of HSP nephritis in childrenolder than 6 y, the development ofthe nephrotic syndrome, and thepresence of crescent formation inmore than 50% of the glomeruli. Arenal biopsy can help to establish thediagnosis and determine prognosis.

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We would like to thank Drs Sam Weinbergand William Brock for the photographs ofthe skin lesions and Dr John Leonidas forthe radiographs.



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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