A Case of Bullous Amyloidosis

  • NAMBA Chika
    Department of Dermatology, Ehime University Graduate School of Medicine
  • MIYAWAKI Saori
    Department of Dermatology, Ehime University Graduate School of Medicine
  • MURAKAMI Shinji
    Department of Dermatology, Ehime University Graduate School of Medicine
  • HASHIMOTO Koji
    Department of Dermatology, Ehime University Graduate School of Medicine
  • SAYAMA Koji
    Department of Dermatology, Ehime University Graduate School of Medicine
  • JOUTOKU Masanori
    Department of Integrated Medicine and Informatics, Ehime University Graduate School of Medicine
  • HIGAKI Jitsuo
    Department of Integrated Medicine and Informatics, Ehime University Graduate School of Medicine
  • MIYAZAKI Tatsuhiko
    Department of Pathology, Division of Pathogenomics , Ehime University Graduate School of Medicine
  • HOSHII Yoshinobu
    Department of Pathology, Yamaguchi University Graduate School Medicine
  • SUGIURA Keisuke
    Fukuonji Clinic

Bibliographic Information

Other Title
  • Bullous Amyloidosis の 1 例
  • ショウレイ Bullous Amyloidosis ノ 1レイ

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Description

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.

Journal

  • Nishi Nihon Hifuka

    Nishi Nihon Hifuka 74 (4), 387-390, 2012

    Western Division of Japanese Dermatological Association

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