Journal of Indian Academy of Oral Medicine and Radiology (Jan 2009)

Ectodermal dysplasia

  • Sonia Saggoo,
  • Anita Munde,
  • Manjula Hebbale,
  • Manjiri Joshi

DOI
https://doi.org/10.4103/0972-1363.57891
Journal volume & issue
Vol. 21, no. 2
pp. 76 – 78

Abstract

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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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