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The Merck Manual--Second Home Edition logo
 
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Chapter 209. Blistering Diseases
Topics: Introduction | Pemphigus | Bullous Pemphigoid | Dermatitis Herpetiformis
 
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Bullous Pemphigoid

Bullous pemphigoid is an autoimmune disease that causes blistering of the skin.

Bullous pemphigoid tends to occur mainly in older people. It is a less serious disease than pemphigus, is rarely fatal, and does not result in widespread peeling of skin. It can involve a large portion of the skin, however, and can be very uncomfortable.

In bullous pemphigoid, the immune system forms antibodies directed against the skin, resulting in large, tense, very itchy blisters surrounded by areas of red, inflamed skin. Blisters in the mouth are uncommon and are not severe. The areas of skin that are not blistered appear normal.

Diagnosis and Treatment

Doctors usually recognize bullous pemphigoid by its characteristic blisters. However, it is not always easy to distinguish from pemphigus and other blistering conditions, such as severe poison ivy; it is diagnosed with certainty by examining a sample of skin under a microscope (skin biopsy). Doctors differentiate bullous pemphigoid from pemphigus by noting the layers of skin involved and the particular appearance of the antibody deposits.

Mild bullous pemphigoid sometimes resolves without treatment, but resolution usually takes months or years. Therefore, most people receive drug therapy. Nearly everyone responds quickly to high-dose corticosteroids, which are tapered after several weeks. Sometimes azathioprine or cyclophosphamide is given as well. Immune globulin given intravenously is a safe, promising new treatment, especially for people who do not respond to conventional drug therapy. Although some local skin care may be needed, most people do not require hospitalization or intensive skin care treatment.

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