Henoch-Schönlein 紫斑病

• 平均年齢: 6歳
• IgA1の血管壁及び腎臓への付着
• 盛り上がった紫斑 "palpable purpura" が特徴

検査

• CBC, 一般生化学
• IgG, IgA, IgM, IgE
• 便ヘモグロビン
• PT, APTT, Fibなど
• 凝固活性(第13因子)
  • ちっちゃいスピッツなら併せて2本は必要
• ASO, ASK
• 尿検査

診断

• CLASSIFICATION CRITERIA FOR HENOCH-SCHÖNLEIN PURPURA ¹

AMERICAN COLLEGE OF RHEUMATOLOGY CLASSIFICATION CRITERIA

• Two of the following criteria must be present:
  • Palpable purpura
  • Age at onset ≤20 yr
  • Bowel angina (postprandial abdominal pain, bloody diarrhea)
  • Biopsy demonstrating intramural granulocytes in small arterioles and/or venules

EUROPEAN LEAGUE AGAINST RHEUMATISM/PEDIATRIC RHEUMATOLOGY EUROPEAN SOCIETY CRITERIA

• Palpable purpura (in absence of coagulopathy or thrombocytopenia) and one or more of the following criteria must be present:
  • Diffuse abdominal pain
  • Arthritis or arthralgia
  • Biopsy of affected tissue demonstrating predominant immunoglobulin A deposition

治療

Treatment of HSP is supportive, with an emphasis on assuring adequate hydration, nutrition, and analgesia. Controversy continues concerning the appropriate use of glucocorticoids in the management of HSP, but steroids are most often used to treat significant gastrointestinal involvement or other life-threatening manifestations. Empiric use of prednisone (1 mg/kg/day for 1 to 2 wk, followed by taper) reduces abdominal and joint pain but does not alter overall prognosis nor prevent renal disease ²

紫斑の鑑別

• 外傷によるもの
• 電撃性紫斑病 (髄膜炎菌感染によるものが最も多い)
• Henoch-Schönlein 紫斑病
• 薬剤性紫斑病 : サルファ剤、ペニシリン、抱水クロラール、フェニトイン
• 新生児電撃性紫斑病 (プロテインC or S 欠損)
• ビタミンC欠乏症
• Ehlers-Danlos syndrome
• ITP
• HUS/TTP
• DIC
• リンパ腫骨髄浸潤
• NAIT(新生児免疫性血小板減少症)
• 先天性・後天性血小板機能不全
• 凝固異常(血友病, vWFなど)