Pemphigus

ABSTRACT

Pemphigus is a group of organ-specific autoimmune disorders with an established immunologic basis. The presence of intraepithelial blisters and erosions of the skin and variable involvement of the mucous menbranes characterize its three major variants, pemphigus vulgaris, pemphigus follaceus, 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 optional quality of life under treatment. this can be achived by the appropriate use of steroid-spating agents. This article addresses the comprehensive management of patients with pemphigus