Bullous pemphigoid

Definition
Bullous pemphigoid is a skin disorder characterized by large blisters.

Causes, incidence, and risk factors
The blisters of bullous pemphigoid usually appear on the areas of the body that flex or move (flexural areas). About one-third of persons with bullous pemphigoid also develop blisters in the mouth. The disorder may occur in various forms, from no symptoms, to mild redness and irritation, to multiple blisters. The cause is not known, but may be related to immune system disorders.

Bullous pemphigoid typically occurs in elderly persons and is rare in young people. It is characterized by a pattern of exacerbations and remissions. In most patients, the disease spontaneously clears within 6 years.

Symptoms

     
  • bullae (large blisters, thin walled sacs filled with clear fluid, greater than 1 cm. diameter)       o multiple       o usually located on the arms, legs, or trunk       o may also occur in the mouth       o may weep, crust over       o may appear deep below the surface of the skin       o may erode the skin, form ulcers or open sores  
  • itching  
  • rashes  
  • mouth sores  
  • bleeding gums

Signs and tests
A skin lesion biopsy shows a characteristic blistering pattern. A special type of skin biopsy may also be performed and examined by direct immunofluorescence. Some physicians may additionally order blood tests to help confirm the diagnosis.

Treatment
Treatment is focused on relief of symptoms and prevention of infection.

Corticosteroids are the treatment of choice. They are given by mouth or by injection. Topical (applied to a localized area of the skin) corticosteroids may also be used on early, localized lesions.

Other medications may include chemotherapy or immunosuppressive medications such as methotrexate, azathioprine, mycophenolate, or cyclophosphamide. Antibiotics in the tetracycline family may be useful in mild cases. Niacin (a B complex vitamin) is sometimes given along with tetracycline.

Expectations (prognosis)
Bullous pemphigoid usually responds well to treatment. In most patients, all medications may be stopped after several years. In a small group, however, the disease will recur after treatment is stopped.

Complications
Infection of the skin lesions is the most common complication.

Calling your health care provider

Call your physician if you notice unexplained blisters on your skin, or, an itchy rash persists despite home treatment.

Johns Hopkins patient information

Last revised: December 8, 2012
by Armen E. Martirosyan, M.D.

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