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Abstract
Background: Pemphigus vulgaris is a severe and possibly fatal skin disease with blisters and erosions on the oral mucosae and the skin. Objective: To identify the best treatments currently used for pemphigus vulgaris. Methods: The results of previously published studies were summarized. Results/conclusion: The use of systemic steroid therapy is indispensable for the treatment of pemphigus vulgaris. Some patients can be treated only with this therapy; however, other cases do not respond to this regimen. For such intractable pemphigus vulgaris cases, a variety of regimens are used in addition to systemic steroid therapy. These include plasmapheresis, steroid pulse therapy, intravenous immunoglobulin (IVIG), various immunosuppressive agents (such as azathioprine, cyclophosphamide, ciclosporin, mycophenolate mofetil and mizoribine) and anti-CD20 monoclonal antibody (rituximab). When these adjuvant therapies are used, we should always be careful for the severe infectious diseases, except for IVIG. In the most intractable cases, a combination of these adjuvant treatments may be used.