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


 
CASE REPORT Table of Contents   
Year : 2023  |  Volume : 27  |  Issue : 5  |  Page : 75-79
Hypohidrotic ectodermal dysplasia: A rare entity


1 Department of Oral and Maxillofacial Pathology and Microbiology, Ph.D. Scholar Karnavati University, Gandhi Nagar, Gujarat, India
2 Departments of Oral and Maxillofacial Pathology and Microbiology, Aditya Dental College and Hospital, Beed, Maharashtra, India
3 Oral and Maxillofacial Surgery, Aditya Dental College and Hospital, Beed, Maharashtra, India
4 Department of Orthodontics and Dentofacial Orthopaedics, D. Y. Patil Dental College and Hospital, Dr.D.Y.Patil Vidyapeeth, Pune, Maharashtra, India

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DOI: 10.4103/jomfp.jomfp_72_21

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Hypohidrotic ectodermal dysplasia (HED) is a genetic disorder which involves a complex group of inherited conditions. This condition is characterised by the failed development of two or more ectodermal derived anatomic structure; for example, the skin, hair, nails, teeth, and sweat glands. It is a phenotypically heterogenous group of illnesses including anodontia with conical teeth, generalised spacing, reduced ability to sweat, very less hair growth, etc. The pattern of inheritance of HED varies from person to person depending on the type of ectodermal dysplasia (ED). The patterns include X-linked recessive, X-linked dominant, autosomal dominant, autosomal recessive, and spontaneous mutation. There is one such common type of ED more in female than male. It is an X-linked HED (XLHED), characterised by hypohidrosis, sparse hair, and teeth abnormalities. The other type can affect both male and female equally and may be inherited in different ways. The present case is a 21-year-old male who presented with hair, teeth, and sweat gland abnormalities.


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Journal of Oral and Maxillofacial Pathology | Published by Wolters Kluwer - Medknow
Online since 15th Aug, 2007