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伴有幽门狭窄的交界性大疱性表皮松解症,其电子显微镜检查结果提示单纯性大疱性表皮松解症。

Junctional epidermolysis bullosa with pyloric stenosis presenting with electron microscopic findings suggestive of epidermolysis bullosa simplex.

作者信息

Wasel N, Idikio H, Lees G, Krol A, Lin A N

机构信息

Division of Dermatology and Cutaneous Sciences, University of Alberta, Edmonton, Canada.

出版信息

Pediatr Dermatol. 2000 Sep-Oct;17(5):395-8. doi: 10.1046/j.1525-1470.2000.017005395.x.

DOI:10.1046/j.1525-1470.2000.017005395.x
PMID:11085671
Abstract

We present an infant girl who was born with pyloric stenosis and epidermolysis bullosa (EB). Electron microscopy of a skin biopsy specimen showed findings suggestive of EB simplex, but immunofluorescence (IF) mapping of the same specimen established the diagnosis of junctional EB. Because electron microscopy findings may sometimes be misleading, an EB patient with pyloric stenosis and electron microscopy findings suggestive of EB simplex should have a biopsy specimen examined by immunofluorescence mapping, which may confirm that the patient in fact has junctional EB.

摘要

我们报告了一名患有幽门狭窄和大疱性表皮松解症(EB)的女婴。皮肤活检标本的电子显微镜检查结果提示为单纯型EB,但同一标本的免疫荧光(IF)图谱确定诊断为交界型EB。由于电子显微镜检查结果有时可能会产生误导,对于患有幽门狭窄且电子显微镜检查结果提示为单纯型EB的EB患者,应通过免疫荧光图谱对活检标本进行检查,这可能会证实该患者实际上患有交界型EB。

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