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Pronunciations azathioprine bullous pemphigoid corticosteroid cyclophosphamide dermatitis herpetiformis immunoglobulin pemphigoid pemphigus

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

• Bullous pemphigoid occurs when the immune system attacks the skin and causes blistering.
• People develop large, itchy blisters with areas of inflamed skin.
• Doctors can diagnose bullous pemphigoid by examining skin samples under a microscope and checking for certain antibody deposits.
• Treatment involves corticosteroids and drugs that suppress the immune system.

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, with itchiness often the first sign of the disease.

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 it from pemphigus vulgaris and other blistering conditions, such as severe poison ivy. Bullous pemphigoid is diagnosed with certainty by examining a sample of skin under a microscope (skin biopsy). Doctors differentiate bullous pemphigoid from pemphigus vulgaris by noting the layers of skin involved and the particular appearance of antibody deposits.

Bullous Pemphigoid

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. The combination of nicotinamide and minocycline Some Trade Names
MINOCIN
or tetracycline Some Trade Names
SUMYCIN
is sometimes successful. Sometimes azathioprine Some Trade Names
IMURAN
or cyclophosphamide Some Trade Names
LYOPHILIZED CYTOXAN
is given as well for more severe disease. Immunoglobulin 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.

Last full review/revision September 2008 by Julie E. Russak, MD