A blistering disease caused by an autoimmune reaction toward proteins in the skin. Typically, men or women between 40 and 60 years old are affected. Painful and itchy blisters and sores may cover a significant portion of the body. Blisters may first appear in the mouth. Diagnosis is made through biopsy and blood tests. Treatment is with oral corticosteroids and drugs that suppress the immune system. Pemphigus vulgaris can lead to a life-threatening skin infection and is fatal if untreated.
The most common form of pemphigus. Blisters develop suddenly and are round or oval, thin walled, tense, and translucent and bilateral in distribution. The lesions have little tendency to heal, and bleed easily when they burst. Since the introduction of corticosteroids, the prognosis for this autoimmune disease is favorable, but the mortality rate is still 5% to 15%. Immunosuppressive agents (e.g., azathioprine or cyclophosphamide) are used with corticosteroid therapy.
Persistent pemphigus. In this variety, blistering sores develop on both the mucous membranes and the skin, and it can be life-threatening when left untreated.