Bullous Pemphigoid

Bullous Pemphigoid, commonly referred to as “BP” -- is a rare, chronic blistering of the skin on the arms, legs or trunk, but can be widespread covering large portions of the body. BP usually affects the elderly. In about one out of five cases, blisters develop inside the mouth and down the throat. BP is usually less serious than pemphigus, and is rarely fatal.

“Bullous” means large blister – so, besides being very itchy, the blisters associated with this skin disease are very large and filled with a clear fluid. The blister sac is thin, so these break very easily.

A biopsy is usually the method most often used to confirm the diagnosis.

Skin itchiness is usually the first sign of the disease. Symptoms can vary from mildly itchy welts to severe blistering. The area affected can be very localized, or widespread. Blisters usually develop in areas on the arms, legs or trunk, but can also appear inside the mouth and over other mucous parts of the body. The blisters have a thin sac, and can weep or break easily, which later form a crusty appearance. Sometimes, after the sacs break, open ulcers can develop which presents the opportunity for secondary infections to develop.

Causes

Bullous pemphigoid is caused by an immune system dysfunction where antibodies are created that attack a thin layer of connective skin tissue, also referred to as the “basement membrane”. The presence of other autoimmune system problems (i.e. diabetes, rheumatoid arthritis) and age, coupled with genetic predisposition can increase the likelihood of developing BP. Additional factors that could possibly contribute to the disease triggers include certain classes of drugs (i.e. furosemide, nonsteroidal anti-inflammatory, captopril, penicillamine and other antibiotics), physical trauma (e.g. radiation, heat or sun burns) and preexisting skin diseases (e.g. lichen planus and psoriasis).

Treatment

Treatment usually focuses on relief of the itching and blistering, and prevention of secondary infections that can result from the open sores (once the blisters pop). For mild to moderate cases, antibiotics (e.g. tetracycline, minocycline) can be used with prescription strength topical steroid creams to provide fairly rapid relief.

Severe cases usually require oral steroids (i.e. prednisone) and are usually best treated in the hospital. A high dose is started and then is slowly tapered down over months or years after blisters stop appearing. Some physicians prefer to use smaller doses of oral steroids, but couple these with immunosuppressives, such as azathioprine or cyclophosphamide.

One last course of action that has achieved recent success for severe cases that don’t respond well to conventional drug therapies is intravenous treatment with immunoglobulin.

In most cases, BP clears up after a few years and treatment can be discontinued. It can completely disappear for several years, and then suddenly reoccur.

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