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[胫前大疱性表皮松解症。单纯型大疱性表皮松解症的一种罕见形式]

[Pretibial epidermolysis bullosa. A rare form of epidermolysis bullosa simplex].

作者信息

Tsianakas P, Fraitag S, Archimbaud A, Verola O, Paul C, Prost C

机构信息

Polyclinique de Dermatologie, Hôpital Saint-Louis, Paris.

出版信息

Ann Dermatol Venereol. 1998 Jan;125(1):34-6.

PMID:9747205
Abstract

INTRODUCTION

Pretibial epidermolysis bullosa had been classified as a rare localized form of autosomal dominant dystrophic epidermolysis bullosa.

OBSERVATION

We report a sporadic case of a patient suffering from bullous lesions induced by minor trauma on pretibial skin. The lesions healed with atrophic scars. No milia formation was observed. The mapping of dermoepidermal junction by LH 7:2 and GB3 monoclonal antibodies was normal. By electron microscopy, numerous perinuclear vacuoles were observed and the cleavage occurred within the basal keratinocytes.

DISCUSSION

This patient had clinical features in accordance with a diagnosis of pretibial epidermolysis bullosa. However, in contrast to previous case reports, the ultrastructural pattern was this of an epidermolysis bullosa of simplex type.

摘要

引言

胫前大疱性表皮松解症曾被归类为常染色体显性营养不良性大疱性表皮松解症的一种罕见局限性类型。

观察

我们报告了一例散发性病例,患者胫前皮肤因轻微创伤出现大疱性皮损。皮损愈合后留下萎缩性瘢痕。未观察到粟丘疹形成。用LH 7:2和GB3单克隆抗体对真皮表皮连接处进行定位显示正常。电子显微镜检查发现大量核周空泡,且裂隙发生在基底角质形成细胞内。

讨论

该患者的临床特征符合胫前大疱性表皮松解症的诊断。然而,与既往病例报告不同的是,超微结构模式为单纯型大疱性表皮松解症。

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