Pemphigus vulgaris is a rare, severe autoimmune disease in which blisters of varying sizes break out on the skin, the lining of the mouth, the genitals, and other mucous membranes.

• Pemphigus vulgaris occurs when the immune system mistakenly attacks proteins in the upper layers of the skin.
• People experience severe blistering in the mouth and on other areas of the body, and sometimes sheets of skin peel off.
• Doctors can diagnose pemphigus vulgaris by examining skin samples under a microscope.
• Treatment usually involves corticosteroids or drugs that suppress the immune system.

Pemphigus develops most often in middle-aged or older people. It rarely develops in children. In this disease, the immune system produces antibodies that attack specific proteins that connect the epidermal cells (the cells in the top layer of skin) to each other. When these connections are disrupted, the cells separate from each other and from the lower layers of the skin, and blisters form. Similar-appearing blisters occur with a less dangerous skin disorder, bullous pemphigoid.

Symptoms

The major symptom of pemphigus vulgaris is the development of clear, soft, usually painless (but sometimes itchy and tender) blisters of various sizes. In addition, the top layer of skin may detach from the lower layers in response to slight pinching or rubbing, causing it to peel off in sheets and to leave painful erosions.

Pemphigus

The blisters often first appear in the mouth and soon rupture, forming painful sores (ulcers). More blisters and ulcers may follow until the entire lining of the mouth is affected, causing difficulty swallowing. Blisters form on the skin as well. These blisters then rupture, leaving raw, painful, crusted wounds. The person feels generally ill. Blisters may be widespread, and once ruptured, they may become infected. When severe, pemphigus vulgaris is as harmful as a serious burn. Similar to a burn, the damaged skin oozes large amounts of fluid and is prone to infection by many types of bacteria.

Did You Know... Pemphigus vulgaris is usually fatal without treatment, but 90% of people survive if they receive treatment.

Diagnosis and Treatment

Doctors usually recognize pemphigus vulgaris by its characteristic blisters, but the disorder is diagnosed with certainty by examining a sample of skin under a microscope (skin biopsy). Sometimes doctors use special chemical stains that allow antibody deposits to be seen under the microscope. Doctors differentiate pemphigus vulgaris from bullous pemphigoid by noting the layers of skin involved and the particular appearance of antibody deposits.

Without treatment, pemphigus vulgaris is usually fatal. With treatment, 90% of people survive. High doses of corticosteroids are the mainstay of treatment. If the disease is controlled, the dose of corticosteroids is tapered. If the person does not respond to treatment or the disease flares up as the dose is gradually reduced (tapered), an immunosuppressant, such as azathioprineSome Trade Names
IMURAN
, cyclophosphamideSome Trade Names
LYOPHILIZED CYTOXAN
, or rituximabSome Trade Names
RITUXAN
, is also given. People with severe pemphigus vulgaris may also undergo plasmapheresis, a process in which antibodies are filtered from the blood (see see Blood Transfusion: Controlling Diseases by Purifying the Blood). Immune globulin given intravenously is a new, safe and effective treatment for severe pemphigus vulgaris. Some people respond well enough to discontinue drug therapy, whereas others must continue taking low doses of the drugs for long periods.

In a hospital, the raw skin surfaces require extraordinary care, similar to the care given to people with severe burns. Antibiotics may be needed to treat infections in ruptured blisters. Dressings, sometimes coated with petroleum jelly, can protect raw, oozing areas.

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