International journal of Medical and Dental Sciences

Ectodermal Dysplasia: Familial Report of Six Cases


Affiliations

  • KIMSDU, Department of Oral Medicine and Radiology, Karad, Maharashtra, India
  • MIDSR Dental College, Department of Oral Medicine and Radiology, Latur, Maharashtra, India
  • KIMSDU, Department of Periodontology, Karad, Maharashtra, India
  • Kaling Institute of Dental Sciences, Department of Periodontology, Bubaneshwar, Orissa, India
  • MIDSR Dental College, Department of Periodontology, Latur, Maharashtra, India
  • Bharthi Vidyapeeth Dental College, Department of Orthodontics, Sangli, Maharashtra, India

Abstract

Ectodermal dysplasia is a hereditary disease characterized by a congenital dysplasia of one or more ectodermal structures and their accessory appendages. There are two main types, Hypohidrotic/Christ-Seimens-Tourian Syndrome and Hidrotic/Clouston syndrome. Common manifestations include defective hair follicles and eyebrows, frontal bossing with prominent supraorbital ridges, nasal bridge depression and protuberant lips. Intraorally common findings are anodontia or hypodontia and conical shaped teeth. The patient may suffer from dry skin, hyperthermia and unexplained high fever because of the deficiency of sweat glands. The present article reports unique case series of ectodermal dysplasia cases in two families, where three generations in the both the families were affected.

Keywords

Christ-Siemens-Touraine Syndrome, Ectodermal Dysplasia, Hypohidrosis, Hypotrichosis, Oligodontia.

Subject Discipline

Dentistry

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