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直接免疫荧光检查阴性的获得性大疱性表皮松解症

Epidermolysis bullosa acquisita with negative direct immunofluorescence.

作者信息

Lacour J P, Juhlin L, el Baze P, Ortonne J P

出版信息

Arch Dermatol. 1985 Sep;121(9):1183-5.

PMID:3899018
Abstract

Epidermolysis bullosa (EB) appeared in a patient at the age of 54 years. Other bullous disorders could be excluded by electron microscopy, and there was no family history of EB. The patient would therefore best be classified as having EB acquisita. Repeated direct immunofluorescence studies were, however, negative for all tested serum samples, suggesting that there might be a subgroup lacking immunoglobulin deposits in the skin. Collagen IV, laminin, and fibronectin were expressed normally at the dermoepidermal junction.

摘要

一名54岁患者出现了大疱性表皮松解症(EB)。通过电子显微镜可排除其他大疱性疾病,且该患者无EB家族史。因此,该患者最适合归类为获得性EB。然而,反复的直接免疫荧光研究对所有检测的血清样本均为阴性,提示可能存在一个皮肤中缺乏免疫球蛋白沉积的亚组。IV型胶原蛋白、层粘连蛋白和纤连蛋白在真皮表皮交界处正常表达。

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