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Hypohidrotic Ectodermal Dysplasia

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hypohidrotic ectodermal dysplasia.
Christ-Siemens-Touraine Syndrome
An X-linked [MIM 305100] ectodermal dysplasia with anhidrosis, heat intolerance, and hypoplasia of sebaceous and sweat glands, resulting in dry, smooth and glossy skin; CSTS is accompanied by gonadal hypoplasia, ageusia, anosmia, URIs, absent nipples, partial anodontia or peg teeth, hypotrichosis, saddle nose, dysphagia, physical and mental retardation, feminine appearance and cleft palate; almost all patients are male

hypohidrotic ectodermal dysplasia (hī´pōhīdrot´ik ektōdur´ml dis-plā´zh),
n a syndrome consisting of hypodontia, hypotrichosis, hypohidrosis, and other defects related to the development of ectodermal structures.


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[FIGURES 1-2 OMITTED] Common Types of Ectodermal Dysplasia The earliest type of ED to be recognized in the world's literature was hypohidrotic ectodermal dysplasia (HED).
12) In 1991, Baran et al (13) published an illustration of dolichonychia from a patient with hypohidrotic ectodermal dysplasia.
Congenitally missing teeth are seen in Witkop syndrome, Book syndrome, hypohidrotic ectodermal dysplasia, incontinentia pigmenti, Ellis-van Creveld syndrome, Hallermann-Streiff syndrome, focal dermal hypoplasia, lipoid proteinosis, Rieger syndrome, otodental dysplasia, Coffin-Lowry syndrome, trichodental syndrome and others.
 
 
 
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