Familial epidermolysis bullosa acquisita.

Epidermolysis bullosa acquisita (EBA) is an acquired blistering skin disorder caused by IgG autoantibodies directed against type VII collagen. In contrast to the genetic forms of epidermolysis bullosa, EBA is usually an acquired, sporadic disease. In this report, we describe a family with two cases of EBA in an uncle-nephew pair, and a third family member with asymptomatic circulating anti-type VII collagen antibodies. These findings provide support for the hypothesis that there is a genetic component to EBA.