부산대학교 도서관

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.