Epidermolysis bullosa acquisita is a chronic autoimmune mucocutaneous disease causing blistering and skin fragility.
Epidermolysis bullosa acquisita usually appears in adults. Bullous lesions may develop on normal-appearing skin spontaneously or may be caused by minor trauma. The trauma-prone areas of the skin, such as the extensor surfaces of elbows, knees, ankles, and buttocks, are most commonly affected. Pain and scarring are common. Because the hands and feet are often involved, disability can be significant. Occasionally, mucosa of eyes, mouth, or genitals is involved. Laryngeal and esophageal involvement also occurs. Diagnosis is by skin biopsy. Lesions respond poorly to corticosteroids. Mild disease may be treated with colchicine, but more severe disease may require cyclosporine or immune globulin.
Last full review/revision September 2008 by Julie E. Russak, MD