Pemphigus vulgaris

Definition of Pemphigus vulgaris

Pemphigus vulgaris is an autoimmune disorder that involves blistering and sores (erosions) of the skin and mucus membranes.

Causes, incidence, and risk factors

Pemphigus is an autoimmune disorder. The immune system produces antibodies against specific proteins in the skin and mucus membranes. These antibodies break the bonds between skin cells. This leads to the formation of a blister. The exact cause is unknown.

Symptoms

About 50% of people with this condition first develop painful blisters and sores in the mouth, followed by skin blisters. Skin sores may come and go.

Signs and tests

The skin separates easily when the surface of unaffected skin is rubbed sideways with a cotton swab or finger. This is called a positive Nikolsky’s sign.

Treatment

Severe cases of pemphigus may need wound management, similar to the treatment for severe burns. People with this condition may need to stay in a hospital and receive care in a burn unit or intensive care unit.

Expectations (prognosis)

Without treatment, this condition is usually life-threatening. Severe infection is the most frequent cause of death.

Review

Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington, School of Medicine; and Roy Colven, MD, Dermatologist, Associate Professor of Medicine, University of Washington Medical School, Seattle, Washington. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. – 9/11/2010

Pemphigus, vulgaris on the back
Pemphigus, vulgaris - lesions in the mouth

ADAM Medical Encyclopedia

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