학술논문

A Case of Bullous Pemphigoid Successfully Treated with Intravenous Immunoglobulin / 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
Document Type
Journal Article
Source
日本皮膚科学会雑誌 / The Japanese Journal of Dermatology. 2008, 118(5):933
Subject
bullous pemphigoid
corticosteroids
intravenous immunoglobulin
lung cancer
Language
Japanese
ISSN
0021-499X
1346-8146
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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