A Case of Bullous Pemphigoid Successfully Treated with Intravenous Immunoglobulin

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  • Shinkuma Satoru
    Department of Dermatology, Hokkaido University Graduate School of Medicine
  • Tsuji-Abe Yukiko
    Department of Dermatology, Hokkaido University Graduate School of Medicine
  • Tomita Yuki
    Department of Dermatology, Hokkaido University Graduate School of Medicine
  • Natsuga Ken
    Department of Dermatology, Hokkaido University Graduate School of Medicine
  • Ujiie Hideyuki
    Department of Dermatology, Hokkaido University Graduate School of Medicine
  • Abe Riichiro
    Department of Dermatology, Hokkaido University Graduate School of Medicine
  • Akiyama Masashi
    Department of Dermatology, Hokkaido University Graduate School of Medicine
  • Shimizu Hiroshi
    Department of Dermatology, Hokkaido University Graduate School of Medicine

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  • 免疫グロブリン大量静注療法が著効した水疱性類天疱瘡の1例
  • メンエキ グロブリン タイリョウ ジョウチュウ リョウホウ ガ チョコウシタ スイホウセイルイ テンホウソウ ノ 1レイ

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Abstract

Bullous pemphigoid is an autoimmune blistering disease in which patients produce autoantibodies to hemidesmosome-basement membrane zone components. Most bullous pemphigoid patients respond well to systemic corticosteroids, but additional therapies such as pulsed corticosteroid therapy or immunosuppressants are sometimes required in more refractory cases. Intravenous immunoglobulin treatments have been increasingly used to treat a range of autoimmune blistering diseases. In the literature, there are several dozen bullous pemphigoid cases which have been successfully treated with intravenous immunoglobulins. An 83-year-old Japanese female presented with widely disseminating edematous erythema, bullae, and erosions that developed over her entire body. Based upon the clinical, histological and immunohistological findings and BP180 ELISA test scores, she was diagnosed with bullous pemphigoid. She was initially treated with prednisolone (0.75 mg/kg/day), but she was resistant to the therapy. Because she also suffered from lung cancer and her general condition was poor, intravenous immunoglobulin therapy (2 g/kg) was performed, resulting in rapid improvement.

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