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...
Main Authors: | , , , |
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Format: | Article |
Language: | English |
Published: |
Wolters Kluwer Medknow Publications
2009-01-01
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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 |
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. |
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ISSN: | 0972-1363 0975-1572 |