Ectodermal dysplasia

Hereditary hypohidrotic ectodermal dysplasia, also called the Christ-Siemens-Touraine Syndrome is characterized by congenital dysplasia of one or more ectodermal structures and is manifested by hypohidrosis, hypotrichosis and hypodontia. It is usually an X-linked recessive mendelian character which...

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Bibliographic Details
Main Authors: Sonia Saggoo, Anita Munde, Manjula Hebbale, Manjiri Joshi
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2009-01-01
Series:Journal of Indian Academy of Oral Medicine and Radiology
Subjects:
Online Access:http://www.jiaomr.in/article.asp?issn=0972-1363;year=2009;volume=21;issue=2;spage=76;epage=78;aulast=Saggoo
Description
Summary:Hereditary hypohidrotic ectodermal dysplasia, also called the Christ-Siemens-Touraine Syndrome is characterized by congenital dysplasia of one or more ectodermal structures and is manifested by hypohidrosis, hypotrichosis and hypodontia. It is usually an X-linked recessive mendelian character which is rarely seen in males. It results from abnormal morphogenesis of cutaneous and oral embryonic ectoderm. Patients with this disorder exhibit smooth , thin and dry skin, fine and blond scanty hair. Intra-orally anodontia or hypodontia, with impaired development of alveolar process is seen. A case report of a rare case of this disorder in a female patient aged 18 years is hereby presented.
ISSN:0972-1363
0975-1572