Misha Rosenbach, MD, Section Editor
Bullous pemphigoid is an autoimmune disease, which means that the cells in the body that normally fight infection attack the body instead. The body's immune system is confused and makes an antibody (type of protein used to fight infection) that targets a part of the skin that normally holds it together. The attack on the skin causes blisters (firm, fluid-filled bubbles on the skin) to form. This disease most often involves only the skin, but the eyes, mouth, and genitals also can be affected. In most cases, the disease develops on its own, but certain medications also can cause bullous pemphigoid to develop.
Bullous pemphigoid commonly affects people older than 60 years but can occur in younger people. Once someone is diagnosed as having this disease, they can have it for many years. Treatment helps to control the disease, but there is no permanent cure.
Severe itching and blisters occur in almost all patients. Early in the course of the disease, some patients may not have blisters but instead have only a rash that looks similar to hives. These hivelike spots can be all over the body; many times, when blisters appear, they will appear on top of this rash. Blisters will sometimes break, and the exposed skin can be raw and painful. Scars usually do not develop, and the skin can return to normal, although darker spots may remain after the blisters go away. You cannot spread it to people around you (it is not contagious).
Your physician will get information from you, look at your skin, take blood, and perform skin biopsies. A skin biopsy requires a small needle injection of numbing medicine (anesthesia) and then removal of a small piece of skin (often about the size of a pencil eraser).
Treatments help control the itch and blisters and help the skin heal. The treatment will depend on how much of your skin is involved. If only a small area of skin is affected, creams applied to the skin may be enough. If a large area is involved, then oral steroids, a medicine that suppresses the immune system, and other medicines are necessary. Oral steroids help the skin but can have a number of adverse effects that require monitoring.
International Pemphigus and Pemphigoid Foundation http://www.pemphigus.orgBritish Association of Dermatologists http://www.bad.org.uk/site/852/default.aspx
To find this and other JAMA Dermatology Patient Page articles, go to the JAMA Dermatology Patient Page link on theJAMA Dermatology website at http://www.jamaderm.com.
Source: International Pemphigus and Pemphigoid Foundation and British Association of Dermatologists
Rosenbach M, Wanat KA, Lynm C. Bullous Pemphigoid. JAMA Dermatol. 2013;149(3):382. doi:10.1001/jamadermatol.2013.112