Pemphigus vulgaris (PV) is a chronic autoimmune intraepithelial blistering disease. PV almost always affects the mouth, and it can be the initial site of presentation in about 40% to 50% of cases, before the skin and other mucosal sites (such as the 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.