Diagnosis and Clinical Features of Epidermolysis Bullosa Acquisita

被引：19

作者：

Caux, Frederic ^{[1
,2
]}

机构：

[1] Univ Paris 13, Avicenne Hosp, AP HP, Dept Dermatol, F-93000 Bobigny, France

[2] Univ Paris 13, Avicenne Hosp, AP HP, Reference Ctr Autoimmune Bullous Dis, F-93000 Bobigny, France

来源：

DERMATOLOGIC CLINICS | 2011年 / 29卷 / 03期

关键词：

Epidermolysis bullosa acquisita; Blistering disease; Collagen VII; Autoimmunity; Anchoring fibrils; Diagnosis; SYSTEMIC-LUPUS-ERYTHEMATOSUS; VII COLLAGEN; IMMUNOELECTRON MICROSCOPY; PASSIVE TRANSFER; SERA REACT; AUTOANTIBODIES; INDUCTION; DOMAIN; AUTOIMMUNITY; ANTIBODIES;

D O I：

10.1016/j.det.2011.03.017

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

Epidermolysis bullosa acquisita (EBA) is a rare autoimmune subepidermal blistering disease characterized by immune deposits on anchoring fibrils of cutaneous and mucosal basement membrane zones. It is due to circulating antibodies directed to type VII collagen. Clinical manifestations include a classical form with skin fragility, blisters and scars on trauma-prone surfaces, an inflammatory form, and a cicatricial pemphigoid-like form. Specialized tests available in only certain laboratories are necessary to confirm a diagnosis of EBA, such as immunoelectron microscopy, immunoblotting, or ELISA using recombinant proteins. A frequent association between EBA and Crohn disease has been observed.

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页码：485 / +

页数：8

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