Christ Siemen Touraine Syndrome - A Case Report

Abstract

Christ Siemen Touraine Syndrome [CST syndrome] more commonly referred to as anhidrotic/hypohidrotic ectodermal dysplasia. It is a hereditary disorder characterized by abnormal development of certain tissues and structures of ectodermal origin. It is diffuse, nonprogressive disease characterized at birth and by involvement of epidermis plus atleast one of appendages (hair, sebaceous glands, nails, teeth or mucous glands). Hypotrichosis, hypodontia, hypohydrosis and occasional onychodysplasia are prominent features. It is an X-linked recessive disorder occurring in its full expression only in males, females as heterozygotes may be mildly affected. A case of 20 year old male with CST syndrome is discussed in this article.

Keywords

• Syndrome
• ectodermal dysplasia.