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儿童获得性大疱性表皮松解症。一名5岁女孩的病例发现。

Childhood epidermolysis bullosa acquisita. Detection in a 5-year-old girl.

作者信息

Rubenstein R, Esterly N B, Fine J D

出版信息

Arch Dermatol. 1987 Jun;123(6):772-6.

PMID:3555356
Abstract

A 5 1/2-year-old girl with a blistering disease involving the skin and the oral, ocular, and anogenital mucosa is described. The initial clinical, histologic, and immunofluorescence findings suggested a diagnosis of cicatricial pemphigoid. However, immunoelectron microscopy demonstrated linear deposits of several immunoreactants within the sub-lamina densa region of the dermoepidermal junction, consistent with the diagnosis of epidermolysis bullosa acquisita. Although epidermolysis bullosa acquisita is considered a disease of adult onset, it should be included in the differential diagnosis of blistering diseases in children.

摘要

本文描述了一名5岁半的女孩,患有累及皮肤、口腔、眼部和肛门生殖器黏膜的水疱性疾病。最初的临床、组织学和免疫荧光检查结果提示诊断为瘢痕性类天疱疮。然而,免疫电子显微镜显示在真皮表皮连接处的致密层下区域有几种免疫反应物的线性沉积,符合获得性大疱性表皮松解症的诊断。尽管获得性大疱性表皮松解症被认为是一种成人发病的疾病,但在儿童水疱性疾病的鉴别诊断中也应予以考虑。

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