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获得性大疱性表皮松解症:通过荧光叠加抗原图谱诊断及对大剂量静脉注射免疫球蛋白的临床反应

Epidermolysis bullosa acquisita: diagnosis by fluorescence overlay antigen mapping and clinical response to high-dose intravenous immunoglobulin.

作者信息

Campos M, Silvente C, Lecona Manuel, Suárez R, Lázaro P

机构信息

Department of Dermatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain.

出版信息

Clin Exp Dermatol. 2006 Jan;31(1):71-3. doi: 10.1111/j.1365-2230.2005.01989.x.

Abstract

Epidermolysis bullosa acquisita (EBA) is a chronic, subepidermal blistering disease characterized by the presence of autoantibodies to type VII collagen, located below the lamina densa of the basement membrane zone (BMZ). There is a large clinical and histological overlap between EBA and other subepidermal autoimmune bullous diseases, therefore, complex immunological techniques are required to make an accurate diagnosis. Therapy of EBA is also a difficult issue. Most patients do not respond to several common immunosuppressive agents. We describe a patient who has shown a good response to high-dose intravenous immunoglobulin therapy.

摘要

获得性大疱性表皮松解症(EBA)是一种慢性的表皮下大疱性疾病,其特征是存在针对位于基底膜带(BMZ)致密层下方的VII型胶原蛋白的自身抗体。EBA与其他表皮下自身免疫性大疱性疾病在临床和组织学上有很大重叠,因此,需要复杂的免疫技术才能做出准确诊断。EBA的治疗也是一个难题。大多数患者对几种常见的免疫抑制剂没有反应。我们描述了一名对大剂量静脉注射免疫球蛋白治疗有良好反应的患者。

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