Bullous Pemphigoid is a rare, autoimmune, chronic skin disorder characterized by blistering. This disorder occurs most frequently in elderly people. Generalized blistering occurs in and under the upper layers of the skin and usually subsides spontaneously within several months or years. However, symptoms may recur. In some rare cases of Bullous Pemphigoid, complications such as pneumonia may develop.
The first symptom of bullous pemphigoid (BP) is usually redness of the skin surrounding a lesion, scar, and/or the navel. Within weeks, thin walled blisters with clear fluid centers (bullae) appear on the undersurfaces of the arms and legs (flexor surfaces), in the armpits (axillae), on the abdomen, and/or around the groin. These blisters are create red, itchy patches. Unlike pemphigus, BP blisters usually do not affect the mucous membrane lining the mouth; if they do they heal rapidly.
The blisters are usually hard and tight, and contain clear or blood-tinged fluid; they do not rupture easily. If the blisters do rupture, pain may occur but healing is usually rapid.
Bullous pemphigoid usually itches and in its early phase, itching and hive-like patches may be the only symptoms.
After a few months, the symptoms of bullous pemphigoid often disappear spontaneously, but they may recur for no apparent reason.
The exact cause of bullous pemphigoid is not known; increasingly, however, it is believed to be an autoimmune disorder. Autoimmune disorders are generated when the body’s natural defenses (e.g., the immune system with its antibodies) against “foreign” or invading organisms, attack healthy tissue for unknown reasons.
Certain drug reactions can produce skin lesions that are very similar to BP. It is essential to determine whether the patient’s symptoms are adverse reactions to the pharmaceuticals, or whether the blisters are the result of an autoimmune reaction.
In 1990 it was determined that the gene for Bullous Pemphigoid is located on chromosome 6 and has been mapped to 6p12-p11.
Bullous pemphigoid is a rare disorder that affects males and females in equal numbers. This disorder primarily affect the elderly.
Doctors with some experience with BP can usually recognize the blisters that characterize the disorder. Others will study one or more skin biopsies to determine the layer(s) of skin involved and the appearance of the antibody deposits between the outer and inner layers of the skin.
Mild cases of bullous pemphigoid may not require any treatment and remission may take place within months or years. More serious bouts of the disorder are treated with steroids, usually prednisone over a longish period of time. Since the cumulative effects of long-term steroid therapy are undesirable treatment aims at the lowest dose over the shortest period of time.
Other medications have been tried with only fair results. These include: tetracycline antibiotics, dapsone, methotrexate, and high dose immuno-globulin.
Corticosteroid drugs are given to people with bullous pemphigoid to help reduce the number of blisters. The dosage of these immune suppressant drugs is lower for people with BP than the dosage prescribed to treat pemphigus. Prednisone can be discontinued in approximately 50 percent of cases of BP because the patients eventually go into remission. The remainder of patients may require maintenance therapy. Because many people with Bullous Pemphigoid are elderly, decisions about whether to treat with drugs that alter the immune system (such as corticosteroids) must be individualized because it may make fragile patients more susceptible to infections.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
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Duke University Medical Center in collaboration with Genentech, a biotechnology company, is sponsoring a Phase I and Phase II clinical trial to measure the safety and efficacy of treating bullous pemphigoid with rituximab, a monoclonal antibody that has been used to treat some cancers.
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