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Pemphigus vulgaris is a prototype antibody-mediated autoimmune disorder in which autoantibodies against desmosomal intraepidermal adhesion proteins lead to acantholysis and blister formation. Although our understanding of the molecular pathogenesis of pemphigus and other autoimmune bullous diseases has increased markedly in recent decades, similar progress regarding effective treatment has not been made.
There are a large number of potential interventions for pemphigus.1 The cornerstone of treatment is corticosteroids, although use of immunosuppressive agents, immunomodulatory agents, plasmapheresis, intravenous immunoglobulin, and biological agents has also been described. The efficacy and role of each of these agents has not been established.
Martin LK, Murrell DF. Treatment of PemphigusThe Need for More Evidence. Arch Dermatol. 2008;144(1):100-101. doi:10.1001/archdermatol.2007.14