International Journal of Medical and Dental Sciences

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Ectodermal Dysplasia: Familial Report of Six Cases


Affiliations
1 School of Dental Sciences, KIMSDU, Karad, Maharashtra, India
2 MIDSR Dental College, Kasargaon, Latur, Maharashtra, India
3 Kaling Institute of Dental Sciences, KIMS Campus, Bubaneshwar, Orissa, India
4 Bharthi Vidyapeeth Dental College, BVPDC Campus, Sangli, Maharashtra, India
 

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.
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  • Ectodermal Dysplasia: Familial Report of Six Cases

Abstract Views: 234  |  PDF Views: 120

Authors

S. R. Ashwani Rani
School of Dental Sciences, KIMSDU, Karad, Maharashtra, India
Shobha Bijjaragi
MIDSR Dental College, Kasargaon, Latur, Maharashtra, India
Girish Suragimath
School of Dental Sciences, KIMSDU, Karad, Maharashtra, India
Prasad Kulkarni
Kaling Institute of Dental Sciences, KIMS Campus, Bubaneshwar, Orissa, India
Arun Kulkarni
MIDSR Dental College, Kasargaon, Latur, Maharashtra, India
Anand Nimbal
Bharthi Vidyapeeth Dental College, BVPDC Campus, 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.

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DOI: https://doi.org/10.18311/ijmds%2F2014%2F81303