後天性表皮水疱症におけるアフェレシス療法 : 当院2症例の治療経験(<特集>皮膚疾患とアフェレシス)

概要

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Epidermolysis bullosa acquisita (EBA) is a comparatively rare autoimmune disease producing blisters at the subepidermal area similarly to bullous pemphigoid. Corticosteroid therapy is the first treatment of choice. However, the disease is refractory to such treatment in more than half of patients. Here, we treated 2 patients initially with systemic corticosteroids and then in combination with double filtration plasmapheresis (DFPP), which proved effective in both cases. The efficacy of DFPP has also been reported in 4 other Japanese cases. Therefore, we believe that it is important to immediately start systemic corticosteroids in combination with DFPP when treating EBA.
2008-05-31