Pemphigus vulgaris (PV) is a chronic autoimmune intraepithelial blistering disease. PV almost always affects the mouth and can be the initial site of presentation in about 40% to 50% of cases before skin and other mucosal sites (esophagus, pharynx, larynx, nasal, and genital areas) become involved. The blisters break to form large denuded areas of skin, which can prove fatal if extensive areas are involved. Early recognition of this lesion may prevent delayed diagnosis and inappropriate treatment of a potentially fatal chronic dermatological condition.