Bullous Amyloidosis の 1 例

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タイトル別名
  • A Case of Bullous Amyloidosis
  • ショウレイ Bullous Amyloidosis ノ 1レイ

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A 92-year-old man presented with pruritic erythema, blisters and erosions on forearms and dorsum that had persisted for 1-month. Histology of the abdominal erythema showed a subepidermal blister and moderate infiltration of lymphocytes and eosinophils around the dermal blood vessels. Direct immunofluorescence and tests for circulating autoantibodies to pemphigus and pemphigoid were negative. As he had a nephrotic syndrome, a renal biopsy was performed, which disclosed renal and systemic amyloidosis. Direct fast scarlet staining revealed a small amount of amyloid deposit on the bottom of a blister. The amyloids were positive for anti-immunoglobulin λ chain antibody, and were negative for κ chain antibody, transthyretin and AA. A diagnosis of AL amyloidosis was established. He refused a bone marrow puncture, and serum and urine immunoelectrophoresis showed no M-protein and no Bence-Jones protein. Topical corticosteroid and oral antihistamines were not effective in preventing new skin lesions and pruritus. He refused a dialysis and died of renal failure 4 months after the first visit.

収録刊行物

  • 西日本皮膚科

    西日本皮膚科 74 (4), 387-390, 2012

    日本皮膚科学会西部支部

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