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克里斯蒂-西门子-图赖讷综合征:一例病例报告及文献综述

Christ-siemens-touraine syndrome: a case report and review of the literature.

作者信息

Mokhtari Sepideh, Mokhtari Saeedeh, Lotfi Ali

机构信息

Department of Oral and Maxillofacial Pathology, Dental School of Shahid Beheshti University of Medical Sciences, Tehran 4739, Iran.

出版信息

Case Rep Dent. 2012;2012:586418. doi: 10.1155/2012/586418. Epub 2012 Nov 29.

Abstract

ECTODERMAL DYSPLASIA IS A RARE DISORDER WITH DEFECTS IN TWO OR MORE OF THE FOLLOWING STRUCTURES

the teeth and the skin and its appendages including hair, nails, eccrine, and sebaceous glands. Anhidrotic ectodermal dysplasia is the most common type of disease. This rare disorder, also known as Christ-Siemens-Touraine syndrome, manifests as a triad of hypotrichosis, asteatosis, and anhidrosis. In view of the rarity of this entity, a classical case of anhidrotic ectodermal dysplasia is reported. We have also provided a review of recent investigations in the area of dental abnormalities in this syndrome.

摘要

外胚层发育不良是一种罕见的疾病,在以下两种或更多结构中存在缺陷:牙齿、皮肤及其附属器,包括毛发、指甲、汗腺和皮脂腺。无汗性外胚层发育不良是最常见的疾病类型。这种罕见疾病,也称为克里斯 - 西门斯 - 图赖讷综合征,表现为毛发稀少、皮肤干燥和无汗三联征。鉴于该疾病的罕见性,本文报道了一例典型的无汗性外胚层发育不良病例。我们还对该综合征牙齿异常领域的最新研究进行了综述。

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