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[遗传性大疱性表皮松解症。23例的流行病学及临床分类]

[Hereditary epidermolysis bullosa. Epidemiology and clinical classification of 23 cases].

作者信息

Zahaf A, Mkaouar M, Rebei R

机构信息

Service de Dermatologie-Vénéréologie, Centre Hospitalo-Universitaire de Sfax 3029 Tunisie.

出版信息

Arch Fr Pediatr. 1988 Dec;45(10):795-8.

PMID:3071287
Abstract

The authors report 23 cases of hereditary epidermolysis bullosa (EB). An attempt has been made to classify them. It was based only on the clinical and genetic criteria. The EB simplex has been found in 14 patients, 5 cases were of the Köbner type. The other 9 cases were of the Cockayne-Weber type. EB dystrophica was isolated in 9 cases, 7 cases of which were carriers of the Hallopeau-Siemens form, one case of the pretibial form and one case of Bart syndrome. The frequencies obtained are largely above the values reported in the literature.

摘要

作者报告了23例遗传性大疱性表皮松解症(EB)。已尝试对其进行分类。分类仅基于临床和遗传学标准。14例患者被诊断为单纯型EB,其中5例为科布纳型。另外9例为科凯恩 - 韦伯型。9例为营养不良型EB,其中7例为Hallopeau - Siemens型携带者,1例为胫前型,1例为巴特综合征。所获得的频率大大高于文献报道的值。

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