Pemphigus is a group of organ-specific autoimmune mucocutaneous disorders with an established immunologic basis. The presence of intraepithelial blisters and erosions of the skin and variable involvement of the mucous membranes characterize its three major variants, pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. Prior to the use of corticosteroids in the 1950s, the natural history of pemphigus vulgaris was relentless progression, with a 50% mortality at 2 years, and almost 100% at 5 years. Today, with mortality rates less than 5%, the focus has changed towards reducing corticosteroid side effects and maintaining optimal quality of life under treatment. This can be achieved by the appropriate use of steroid-sparing agents. This article addresses the comprehensive management of patients with pemphigus.
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