自己免疫性水疱症とアフェレシス : 後天性表皮水疱症

NDLデジタルコレクション 被引用文献1件

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  • Autoimmune Bullous Disease and Apheresis : Epidermolysis Bullosa Acquisita

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Epidermolysis bullosa acquisita is a rare autoimmune disease that has been well known to be resistant to many treatments. This disease has two clinical subsets, classical and nonclassical, the latter mimicking bullous pemphigoid in that the eruption spreads over the extensive areas of the body and requires intensive treatments. Although systemic corticosteroids. are the first choice of the treatments in this subset, the eruption tends to relapse with a tapering of doses. So adjuvant therapies such as plasmapheresis or immunosupplessive drugs are usually necessary. To our knowledge, in the literature there have been these cases of epjdermolysis bullosa acquisita in which plasmapheresis produced significant effects and four cases with poor responses. We found that in the latter cases, corticosteroids and/or immunosuppressive drugs were given for a long period, and relapses occurred repeatedly before plasmapheresis was started. We stress that it is important to start the adjuvant therapies as soon as possible when a sign of relapse occurs with a tapering off of corticosteroids. Plasmapheresis may be one useful measure in the treatment of epidermolysis bullosa acquisita, although more cases are needed to clarify the efficacy and its mode of action.

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