Bullous Pemphigoid

• What is bullous pemphigoid?
• What are symptoms of bullous pemphigoid?
• How is it diagnosed?
• How is bullous pemphigoid treated?

What is bullous pemphigoid?

Bullous pemphigoid is a skin disease characterized by tense blistering eruptions on the surface of the skin. Occasionally, the inner lining tissue of the mouth (mucous membrane tissue) can be involved. The condition is caused by antibodies and inflammation abnormally accumulating in a certain layer of the skin or mucous membranes. This layer of tissue is called the "basement membrane." These antibodies (immunoglobulins) bind to proteins in the basement membrane called hemidesmosomal BP antigens and this attracts cells of inflammation.

A majority of cases of bullous pemphigoid occur in people 50 years of age or older. While the cause is unknown, it is felt by some that an aging immune system may become activated in certain individuals with some genetic predisposition to develop bullous pemphigoid.

What are symptoms of bullous pemphigoid?

Symptoms of bullous pemphigoid include intense itching and burning sensation of the skin. When the mucous membranes of the mouth are affected, it can cause pain, burning, and sensitivity to acidic foods. Eating can be difficult, and involvement in the deeper areas of the throat can cause coughing. Involvement of the inner nose can cause nosebleeds.

How is bullous pemphigoid diagnosed?

Bullous pemphigoid is diagnosed based on the results of a biopsy of involved tissue. The biopsy can reveal the abnormal antibodies deposited in the basement membrane layer of skin or mucous membrane tissue.

Bullous pemphigoid-like condition can sometimes be associated with other illnesses, including systemic lupus erythematosus (SLE or lupus) and cancer.

How is bullous pemphigoid treated?

Bullous pemphigoid can be chronic and mild without affecting the general health of affected individuals. Treatment of bullous pemphigoid can resolve with topical cortisone creams but sometimes requires high doses of cortisone ("steroids") taken internally. Severe bullous pemphigoid can also require immune-suppression drugs such as azathioprine (Imuran). Tetracycline has been used as a treatment option. Other treatments that have been used for severe disease include intravenous immunoglobulin infusions, typically given monthly.

Recent research has indicated that large quantities of high-potency topical corticosteroids applied to the entire body surface were safer and more effective in controlling extensive bullous pemphigoid than oral corticosteroids. It was felt by the researchers that topical corticosteroids should now be the treatment of choice for bullous pemphigoid, particularly when the disease is not extensive.

From the Doctors at MedicineNet.com

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