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隐性营养不良型大疱性表皮松解症的电子显微镜诊断

[Diagnosis by electron microscopy of recessive dystrophic epidermolysis bullosa].

作者信息

Glorio R R, Solari A, Woscoff A

机构信息

División Dermatología, Hospital de Clínicas José de San Martín.

出版信息

Medicina (B Aires). 2000;60(3):354-6.

Abstract

Inherited epidermolysis bullosa (EB) includes a number of distinctive diseases that are characterized by the presence of fragile skin and the tendency to develop blisters and erosions. The current classification separates the types of EB on the basis of the ultrastructural level of the blisters. The electron microscopy is very important for the diagnosis and in the recessive dystrophic EB shows that the lamina densa forms the roof of the blister and that the number of anchoring fibrils are absent or reduced. We present the case of a 30 year old woman with a diagnosis of recessive dystrophic EB diagnosed by electron microscopy.

摘要

遗传性大疱性表皮松解症(EB)包括多种独特的疾病,其特征为皮肤脆弱,容易出现水疱和糜烂。目前的分类是根据水疱的超微结构水平来区分EB的类型。电子显微镜检查对诊断非常重要,在隐性营养不良型EB中显示致密板形成水疱的顶部,且锚定原纤维数量缺失或减少。我们报告一例30岁女性患者,经电子显微镜检查诊断为隐性营养不良型EB。

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