Henoch Solein Purpura

VASCULITIS DISEASE OF THE SMALL VESSELS

SKIN, JOINTS, GI TRACT AND KIDNEY

PALPABLE PURPURA, ARTHRITIS/ARTHRALGIA, DIFFUSE ABDOMINAL PAIN, NEPHRITIS

HISTORY

WILLAN AND HEBERDEN (1806) — PALPABLE PURPURA DUE TO VASCULITIS

J.SCHONLEIN (1837) — + JOINTS PAIN

E. HENOCH (1874) — + NEPHRITIS AND GI BLEEDING

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 1996-2000: 4 CASES

AFFECTS ALL AGES, 2 – 11 YEARS OLD, MALE : FEMALE = 1,5 : 1

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 : TESTES, PANCREAS, PAROTID GLAND, MUSCLES, CNS, AND LUNGS

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

AcRA CRITERIA :

PALPABLE PURPURA

AGE OF ONSET ≤ 20 YEARS OLD

BOWEL ANGINA

SKIN BI0PSY : GRANULOSIT (+)

DIAGNOSIS : 2 OF 4 SYMPTOMS→ HSP(+)

DIFFERENTIAL DIAGNOSIS

ACUTE ABDOMINAL PAIN

RHEUMATOID ARTHRITIS

RHEUMATOID FEVER

SPESIFIC LAB TEST FOR HSP (-)

THERAPY : STEROID 1-2 mg/kgBW

PROGNOSIS :

DEPENDS ON THE SEVERITY AND WIDTH OF KIDNEY DAMAGE

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