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HENOCH–
PURPURA
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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 (+)
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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
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
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