Epidermolysis bullosa acquisita: What's new?

摘要: Type VII collagen is an adhesion molecule of the extracellular matrix in epithelial basement membranes, and main constituent anchoring fibrils at dermal-epidermal junction (DEJ). Autoimmunity against this protein causing rare organ-specific epidermolysis bullosa acquisita (EBA). EBA a acquired, heterogeneous, chronic blistering disease skin mucous membranes characterized by subepidermal blisters tissue-bound as well circulating autoantibodies to DEJ. has several distinct clinical presentations with other bullous diseases, such mainly dystrophic or pemphigoid. The immunoglobulin G for react 290-kDa dermal protein, type collagen, detected immunoblot analysis using extracts. pathogenicity these been demonstrated experimental animal models, which anti-type antibodies injected into mouse produced EBA-like animal. cases often require high doses systemic corticosteroids variety immunosuppressants. Although treatment frequently difficult unsatisfactory, some therapeutic success reported colchicine, dapsone, infliximab i.v. immunoglobulin. In review, we will focus on recent progress our understanding manifestations, etiopathogenesis management EBA.