Contributor: Gordon K. Klintworth
Pemphigus vulgaris is an extremely serious chronic blistering cutaneous autoimmune disease caused by antibodies to desmoglein III on the surface of epidermal keratinocytes. The lesions occur most often on the scalp and mucous membranes as well as in the intertriginous and periumbilical regions. Even the eyelid may be involved. The blisters readily rupture and produce denuded or crusted areas. Vescicles form between the stratum spinosum and outer layers of the epidermis and contain acantholytic cells and a moderate number of eosinophils, lymphocytes, macrophages and polymorphonuclear leukocytes. The disorder may occur in all age groups, but it affects mainly persons 30-50 years old. Individuals of Jewish or Mediterranean origin are partricularly susceptible to the pemphigus vulgaris. In the absence of treatment pemphigus vulgaris is a progressive and usually lethal disease. Therapy with corticosteroids and immunosuppressive agents has decreased the 10 year mortality to <10%.
Pemphigus vulgaris needs to be differentiated from other related blistering diseases (pemphigus vegetans, pemphigus foliaceus, pemphigus erythematosus, Brazilian pemphigus foliaceus, drug induced pemphigus, familial benign chronic pemphigus and transient acatholytic dermatosis. Often pemphigus vulgaris is associated with other autoimmune diseases, such as myasthenia gravis and lupus erythematosus.