Pemphigus Foliaceus and Pemphigus Vulgaris

Abstract

Pemphigus is a group of autoimmune disorders characterized by spontaneous intraepidermal blisters and epidermal-specific autoantibodies. Blister formation occurs by the histologically distinctive loss of epidermal cell—cell adhesion, termed acantholysis. The two major forms of the disease are pemphigus vulgaris (PV) and pemphigus foliaceus (PF). In PV, the epidermal-cell separation occurs just above the basal layer of the epidermis, whereas, in PF, the cell separation occurs at the more superficial level of the granular-cell layer of the epidermis. Although PV and PF are diseases described all over the world, there is an endemic form of PF that has been observed in Brazil since the turn of the century and recently in Colombia. This endemic form of PF is also known as “fogo selvagem” (FS; wild fire). Prior to the advent of immunosuppressive therapy, the mortality caused by these disorders was extremely high.KeywordsAutoantibody TiterPemphigus FoliaceusEndemic FormBullous Skin DiseaseCadherin RepeatThese keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.