Bullous Pemphigoid

Bullous pemphigoid

Bullous pemphigoid (BP) is a chronic blistering of the skin. It ranges from mildly itchy welts to severe blisters that are susceptible to infection.

It may affect a small area of the body or be widespread. The majority of those affected are elderly, but it can occur in people of any age.

What Causes Bullous Pemphigoid?

Bullous pemphigoid is an autoimmune disorder, meaning it is caused when the body's own immune system attacks healthy tissue. The immune system produces antibodies to defend the body against bacteria, viruses, and other infections. People with BP produced antibodies that attack certain parts of the skin. Some factors have been shown to play a role in triggering BP. These include drugs (furosemide, penicillin), mechanical trauma, and physical traumas (burns from radiation, sun, or heat).

Bulla is the medical term for a large blister (a thin-walled sac filled with clear fluid). Usually the skin in BP is very itchy, and large, red welts and hives may appear before or during the formation of blisters. The blisters are widespread and usually appear on the areas of the body that flex or move (flexural areas). About 15-20 percent of people with BP also develop blisters in the mouth or down the throat in the esophagus.

How is Bullous Pemphigoid Diagnosed and Treated?

Because BP can appear in many different ways, your doctor may perform a skin biopsy to confirm the diagnosis.

Treatment is focused on relieving symptoms and preventing infection. Oral antibiotics, such as doxycycline or minocycline, may be useful for mild to moderate disease. Their anti-inflammatory effects are used to control the immune system, not to kill bacteria. They can be used in combination with potent topical corticosteroids for more rapid relief.

Oral steroids (prednisone) are the treatment of choice for severe cases. Regular visits will be needed because the dose must be adjusted frequently, and side effects must be monitored. A fairly high dose is needed initially, and once the blisters have stopped appearing, it is slowly reduced over many months or years. As steroids have some undesirable side effects, doctors try to reduce the dose as low as possible. If this is done too quickly, the blisters may reappear.

Often, immunosuppressive agents (Imuran, Cellcept, methotrexate, cyclophosphamide, and Neoral) are combined with the oral steroids to allow a lower dose. Severe cases are best treated in the hospital to allow expert dressing of the wounds and intravenous injections of the most potent treatments.

What Should I Expect if I Have Bullous Pemphigoid?

BP is a self-limiting disease that is in most cases completely clears up over time and the treatment can be stopped. Treatment is usually needed for several years, but generally after a few months it is possible to reduce the dose to reasonably low levels. BP sometimes has a pattern of remissions and flare-ups. It may be dormant for 5 or 6 years, and then suddenly flare.

With careful management, most patients with BP do well. Be patient and follow your instructions faithfully; these are the keys to good results.



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