Epidermolysis bullosa acquisita
作者： Catherine Prost-Squarcioni , Frédéric Caux
DOI: 10.1007/978-3-662-45698-9_40
关键词:
摘要: Epidermolysis bullosa acquisita (EBA) is a rare subepidermal autoimmune bullous disease (AIBD) characterized by immune deposits on anchoring fibrils (AFs) of cutaneous and mucosal basement membrane zones (BMZ). It due to circulating autoantibodies (Ab) directed type VII collagen (C7). Clinical manifestations include classical form with skin fragility, blisters scars trauma-prone surfaces, misleading forms mimicking pemphigoid (BP), mucous (MMP), or linear immunoglobulin (Ig) A (LAD). Prognosis related extent lesions ocular, laryngeal, and/or esophageal involvements. frequent association between EBA Crohn’s has been observed. Specialized tests available in only certain laboratories are necessary confirm its diagnosis such as immunoelectron microscopy (IEM), immunoblotting, ELISA using recombinant proteins. This orphan often misdiagnosed, awareness programs for physicians patients mandatory.
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