Epidermolysis bullosa acquisita.

[dystrophic epidermolysis bullosa]

Epidermolysis bullosa acquisita (EBA ) is a chronic autoimmune subepidermal bullous disease with clinical features similar to the genetic form of dystrophic epidermolysis bullosa . EBA is characterized by the presence of autoantibodies against type VII collagen which is a major component of the anchoring fibrils at the dermal-epidermal junction . EBA can be divided into two main clinical types ; mechanobullous and inflammatory EBA . Mechanobullous EBA , referred to as classic EBA , presents with skin fragility , blisters and dystrophic changes on trauma-prone areas . Inflammatory EBA resembles other autoimmune subepidermal bullous diseases . Compelling evidence from mouse models supports a pathogenic role of autoantibodies against type VII collagen in EBA . Treatment of EBA is often unsatisfactory . The most widely used systemic treatment is corticosteroids . Colchicine and dapsone have been reported to be good treatment modalities when combined with corticosteroids . Some intractable cases of EBA have successfully been treated with intravenous immunoglobulin or rituximab .