Ectodermal dysplasia

Saggoo, Sonia; Munde, Anita; Hebbale, Manjula; Joshi, Manjiri
April 2009
Journal of Indian Academy of Oral Medicine & Radiology;Apr-Jun2009, Vol. 21 Issue 2, p76
Academic Journal
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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