Bullous pemphigoid is an acute or chronic autoimmune skin disease, involving the formation of blisters, more appropriately known as bullae, at the space between the skin layers epidermis and dermis. It is classified as a type II hypersensitivity reaction, with the formation of anti-hemidesmosome antibodies.
In most cases of bullous pemphigoid, no clear precipitating factors are identified. Potential precipitating events that have been reported include exposure to ultraviolet light and radiation therapy. Onset of bullous pemphigoid has also been associated with certain drugs, including furosemide, and other nonsteroidal anti-inflammatory agents, captopril,penicillamine, and antibiotics.
The most common symptom of pemphigoid is blistering that occurs on the arms, legs, abdomen, and mucous membranes. Hives and itching are also common. The blisters have certain characteristics, regardless of where on the body they form: they are often preceded by a red rash, they are large and filled with fluid that is usually clear, but may contain some blood they are thick and do not rupture easily, the skin around the blisters may appear normal or slightly red or dark, ruptured blisters are usually sensitive and painful.
Pemphigoid cannot be cured, but treatments are usually very successful at relieving symptoms. Corticosteroids, either in pill or topical form, will likely be the first treatment your doctor prescribes. These medications reduce inflammation and can help to heal the blisters and relieve itching. However, they can also cause serious side effects, especially from long-term use, so your doctor will take you off of the corticosteroids after the blistering clears up. Another treatment option is to take medication that suppresses your immune system, often in conjunction with the corticosteroids. Immunosuppressants help, but they also put you at risk for other infections. Certain antibiotics, such as tetracycline, may also be prescribed to reduce inflammation and infection.
BP, even without therapy, is often a self-limiting disease, but it may last from several months to many years. About one-half of the treated patients attain remission within 2.5-6 years; however, in individual patients, the disease may continue for 10 years or more. Clinical remission with reversion of DIF and IIF to negative has been noted in patients, even in those with severe generalized disease. Bernard et al. studied the prognostic factors for relapse in the first year after cessation of therapy and concluded that high-titer anti-BP180 ELISA score and, to a lesser degree, a positive DIF finding are good indicators of further relapse of BP. At least one of these tests should be performed before therapy is discontinued. The median length of follow-up for people with bullous pemphigoid was 1.6 (range 0-9) years, and that for pemphigus vulgaris was 2.0 (0-10.4) years. Losses to follow-up were similar in cases and controls in both diseases (by the end of year 2, bullous pemphigoid cases and controls 34% lost; pemphigus vulgaris cases 30% lost, controls 31% lost) .