Epidermolysis bullosa acquisita

Abstract

Epidermolysis bullosa acquisita (EBA) is arare acquired subepidermal bullous autoimmune dermatosis, associated with autoantibodies against collagen typeVII, the most important component of dermal anchoring fibrils. Blister induction occurs after binding of autoantibodies to collagen typeVII, leading to complement activation, recruitment of neutrophils and secretion of proteases. Clinically, the disease is mostly characterized by tense blisters on trauma-exposed body areas which heal with scarring (mechanobullous form of EBA). The second most frequent subtype of EBA is inflammatory EBA, abullous pemphigoid-like disease associated with pruritus. Involvement of mucous membranes and/or lesions in the head and neck area additionally point to the diagnosis of EBA. The mechanobullous type of EBA and EBA with intensive mucous membrane lesions display achronic course and are often extremely resistant to therapy. Topical and systemic glucocorticoids, dapsone, colchicine, classical immunosuppressants, anti-CD20 antibodies, immunoadsorption or intravenous immunoglobulins have been reported as treatments.