HENOCH–SCHONLEIN

HENOCH–
PURPURA

M. Sjabaroeddin Loebis, Lily Irsa, Rita Evalina

Allergy Immunology Division
Pediatrics Departement
Medical Faculty Sumatera Utara University

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INTRODUCTION
HENOCH SCHONLEIN PURPURA / ANAPHYLACTOID
PURPURAE / NON TROMBOSITOPHENIC PURPURAE
→ VASCULITIS DISEASE OF THE SMALL VESSELS
→ SKIN, JOINTS, GI TRACT AND KIDNEY
→ PALPABLE PURPURA,
PURPURA, ARTHRITIS/ARTHRALGIA,
DIFFUSE ABDOMINAL PAIN, NEPHRITIS or HEMATURIA

HISTORY
WILLAN AND HEBERDEN (1806)  PALPABLE PURPURA
DUE TO VASCULITIS
J.SCHONLEIN (1837)  + JOINTS PAIN
E. HENOCH (1874) + NEPHRITIS AND GI BLEEDING
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INCIDENCE
- USA : 14 – 15 CASES/100.000
- ENGLAND : 20,4 CASES/100.000
- NORWAY : 3,3 CASES/100.000
- INDONESIA
RSCM 1998 - 2003 : 23 CASES
RSWS MAKASAR 1996-
1996-2000: 4 CASES

AFFECTS ALL AGES, 2 – 15 YEARS OLD, PEAK :
4-7 YO, MALE : FEMALE = 1,5 : 1

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ETIOLOGY
 Still unknown
 Genetic factor, UTRI, food,immunization,
medicine
 After treatment by antirheumatic, MTX,
anti-TNF
 Increase IgA serum concentration,
immune compleks, and IgA deposit in
vessel walls / mesangial renal 
important role
 PATOFISIOLOGY
 Renal / skin biopsi : immune deposit compleks
(contains IgA)
 Complement activation (alternative pathway)
 Mediator inflamation activation (vascular
prostaglandin)  small vascular inflamation in
the skin, renal, joint and abdominal  skin
purpura, nephritis, arthritis and GIT bleeding
 Histologis : vasculitis leukocitoclastic
 CLINICAL MANIFESTATION
 PALPABLE PURPURA (>75% OF CASES),
ARTHRALGIA OR ARTHRITIS, DIFFUSE
ABDOMINAL PAIN, GI BLEEDING
 KIDNEY DAMAGE → VARIED IN
INCIDENCE AND SEVERITY, 20 – 80 %
 LESION IN OTHER ORGANS : TESTIS,
PANCREAS, PAROTID GLAND, MUSCLES,
CNS, AND LUNGS

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• ARTHRALGIA & ARTHRITIS  68-
68-75 % OF CASES 
SWOLLEN,, PAINFUL, BLEEDING & EFFUSION (-
SWOLLEN (-)

• AcRA CRITERIA :
PALPABLE PURPURA
AGE OF ONSET ≤ 20 YEARS OLD
BOWEL ANGINA
SKIN BI0PSY : GRANULOSIT (+)

DIAGNOSIS : 2 OF 4 SYMPTOMS→ HSP(+)

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Examination
 Lab finding : no specifik
 GIT bleeding : moderate lekocytosis,
normochromic anemia
 Eosinophylia
 ESR : increase
 IgA serum : sometime increase
 Skin biopsy : lekocitoclastik vasculitis
 Immonoflerence : deposit IgA and
complemen on the vessel walls
DIFFERENTIAL DIAGNOSIS OF HSP
ACUTE ABDOMINAL PAIN
RHEUMATOID ARTHRITIS
RHEUMATOID FEVER

SPESIFIC LAB TEST FOR HSP (-

( -)

THERAPY→
THERAPY → STEROID 1-
1-2 mg/kgBW

PROGNOSIS :
DEPENDS ON THE SEVERITY AND WIDTH OF KIDNEY
DAMAGE

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 SIDE EFFECTS OF STEROID

-HYPERTENSION -HIRSUTISM
-HYPERGLYCEMIA -MIOPATHY
-OBESITY -PSEUDOMOTOR CEREBRI
-MOON FACE -CATARACT
-OSTEOPOROSIS -GLAUCOMA
-ACNE -MENTAL RETARDATION

(Am J. DisChild 78:132; 806-

806-10)
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KIDNEY USG : NEPHROPATHY APPEARANCES IN BOTH KIDNEY
NORMAL BLADDER
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First week Second week 13
RECOVERED

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