Bullous pemphigoid (BP) is the most common autoimmune blistering disease in the West. Incidence figures are not available for most parts of the world but BP appears to be rarer in the Far East. Bullous pemphigoid is usually a disease of the elderly but it can also affect younger people and children. Both sexes are similarly affected. While BP usually resolves within five years, there is a moderate death rate associated with the disease and its treatment. Oral corticosteroid drugs are the most common treatment, but may be associated with serious adverse effects, including some deaths. The most common adverse effects of oral steroids, include weight gain and high blood pressure. Long-term use is associated with an increased risk of diabetes mellitus and decreased bone density. Topical steroids are also associated with adverse effects, such as thinning of the skin and easy bruising. The risk of experiencing adverse effects of topical steroids depends on the strength of the steroid, how long it is used for, which area of the body it is applied to, and the kind of skin problem; if a high-strength, potent steroid is used, enough may be absorbed through the skin to cause adverse effects in the rest of the body.
Other treatments include azathioprine, mycophenolate mofetil, dapsone, methotrexate, cyclosporin, cyclophosphamide, plasma exchange, erythromycin, and tetracycline and nicotinamide. Some of these drugs or interventions have the potential for severe adverse effects such as increased susceptibility to serious infections, liver and kidney damage, and bone marrow suppression; and many are very expensive.
Three new studies were included in this update of the review published in 2005 making a total of 10 randomised controlled trials with a total of 1049 participants. All studies involved different comparisons, none had a placebo group. Different doses and formulations of corticosteroids plus azathioprine showed no significant differences in disease control, although azathioprine reduced the amount of prednisone required for disease control. There were no significant differences in healing or disease-free intervals in participants taking azathioprine compared with mycophenolate mofetil, or in disease response comparing tetracycline plus nicotinamide with prednisolone. One small study using Chinese traditional medicine, 'Jingui Shenqi Pill' (JSP), plus prednisone did not show any benefit in favour of adding this traditional Chinese herbal remedy. Most of the deaths were in participants taking high doses of oral corticosteroids.
The review of trials concluded that lower doses of oral steroids and strong steroid creams seem safe and effective. However, the use of steroid creams in extensive disease may be limited by side-effects and the practicality of applying creams to large areas of the skin. Milder regimens of topical steroids are safe and effective in moderate BP. More research is needed on treatments for BP, in particular, the effectiveness of adding plasma exchange, azathioprine or mycophenolate mofetil to corticosteroids, and the treatment with tetracyclines and nicotinamide.