Epidermolysis bullosa acquisita

摘要: EBA is a clinically heterogeneous acquired, subepidermal bullous disease. In its classical form, it mechanobullous disease with skin fragility and trauma-induced blisters that have minimal inflammation heal scarring milia — features are highly reminiscent of hereditary dystrophic forms epidermolysis bullosa. A hallmark IgG autoantibodies targeted against the type VII collagen within anchoring fibrils. Anchoring fibrils structures anchor epidermis underlying BMZ onto dermis. However, has also become evident may present clinical manifestations pemphigoid (BP), cicatricial (CP), Brunsting-Perry pemphigoid. The diagnostic criteria for are: (1) spontaneous or resembling DEB, (2) adult onset, (3) negative family history EB, (4) exclusion all other diseases. Due to pronounced caused by autoantibody-induced paucity fibrils, difficult treat. BP-like inflammatory variants respond well systemic corticosteroids immunosuppressive adjuvants while variant MMP-like do much lesser extent. Novel therapeutic strategies such as immunoadsorption anti-CD20 monoclonal antibody, rituximab, had beneficial effects in small case series.