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An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists


 
ONLINE ONLY ARTICLES - CASE REPORT Table of Contents   
Year : 2019  |  Volume : 23  |  Issue : 3  |  Page : 479
Hypohidrotic ectodermal dysplasia with autosomal recessive inheritance pattern: Report of a rare and unusual case with a brief review of literature


1 Department of Oral Medicine and Radiology, Faculty of Dentistry, Jamia Millia Islamia, New Delhi, India
2 Department of Prosthodontics, Faculty of Dentistry, Jamia Millia Islamia, New Delhi, India
3 Department of Periodontology, Faculty of Dentistry, Jamia Millia Islamia, New Delhi, India
4 Department of Intern, Faculty of Dentistry, Jamia Millia Islamia, New Delhi, India

Correspondence Address:
Madhuri Alankar Sawai
Department of Periodontology, Faculty of Dentistry, Jamia Millia Islamia, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jomfp.JOMFP_98_19

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Ectodermal dysplasia (ED) is a congenital disorder primarily affecting the ectodermal tissue, with infrequent dysfunction of mesodermally derived tissues. Clinically, there are two major forms seen, hypohidrotic/Christ–Siemens–Touraine syndrome and hidrotic/Clouston syndrome, depending on the number and function of sweat glands. A multidisciplinary treatment protocol is usually followed and necessitates collective efforts by medical and dental professionals. Dental intervention should be done as early as possible to ameliorate the patient's esthetics and enhance the emotional and psychological quotient in these patients. This case report aims to highlight a rare and interesting report of hypohidrotic ED in a young female patient with a possible autosomal recessive inheritance pattern.


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Journal of Oral and Maxillofacial Pathology | Published by Wolters Kluwer - Medknow
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