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.
Forty patients with subepidermal autoimmune bullous disorders as well as one patient with pemphigus vulgaris were diagnosed. The incidence of the individual diseases and the age distribution of the patients are depicted By far the most common disease was BP with a total of 27 patients and an incidence of 13.4 newly diagnosed cases per million population and year. Age- and gender-specific incidence of BP. Survival curve demonstrated an early increased mortality: 17% at 3 months and 31% at 6 months, mainly from sepsis and cardiovascular diseases. Of the factors related to bullous pemphigoid activity (duration; pruritus; and number and extent of blisters, eosinophilia, and serum antibodies) only generalized pemphigoid was predictive of death in comparison with localized forms. In multivariate analysis, age of 86 years or more (relative risk, 7.1; 95% confidence interval [CI], 2.0-25.4; P≤.01), poor general condition (relative risk, 8.2; 95% CI, 3.0-22.4; P≤.001), female sex (relative risk, 2.4; 95% CI, 1.1-5.4; P≤.05), and generalized disease (relative risk, 4.4; 95% CI, 1.4-13.7; P≤.01) were associated with increased risks of death at 6 months.