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- W2078967558 abstract "SummaryEBA is a clinically heterogeneous acquired, subepidermal bullous disease. In its classical form, it is a mechanobullous disease with skin fragility and trauma-induced blisters that have minimal inflammation and heal with scarring and milia — features that are highly reminiscent of hereditary dystrophic forms of epidermolysis bullosa. A hallmark of EBA are IgG autoantibodies targeted against the type VII collagen within anchoring fibrils. Anchoring fibrils are structures that anchor the epidermis and its underlying BMZ onto the dermis. However, it has also become evident that EBA may present with clinical manifestations reminiscent of bullous pemphigoid (BP), cicatricial pemphigoid (CP), and Brunsting-Perry pemphigoid. The diagnostic criteria for EBA are: (1) spontaneous or trauma-induced blisters resembling hereditary DEB, (2) adult onset, (3) a negative family history for EB, and (4) the exclusion of all other bullous diseases. Due to the pronounced skin fragility caused by the autoantibody-induced paucity of anchoring fibrils, EBA is difficult to treat. The BP-like inflammatory variants of EBA respond well to systemic corticosteroids and immunosuppressive adjuvants while the mechanobullous variant and the MMP-like variants do to a much lesser extent. Novel therapeutic strategies such as immunoadsorption and the anti-CD20 monoclonal antibody, rituximab, had beneficial effects in small case series." @default.
- W2078967558 created "2016-06-24" @default.
- W2078967558 creator A5007855326 @default.
- W2078967558 creator A5027358797 @default.
- W2078967558 creator A5031646241 @default.
- W2078967558 creator A5065412207 @default.
- W2078967558 date "2011-01-01" @default.
- W2078967558 modified "2023-09-28" @default.
- W2078967558 title "Epidermolysis Bullosa Acquisita" @default.
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- W2078967558 doi "https://doi.org/10.1007/978-3-211-99225-8_6" @default.
- W2078967558 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/3153447" @default.
- W2078967558 hasPublicationYear "2011" @default.
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