Year: 2018 | Month: September | Volume: 8 | Issue: 9 | Pages: 281-286
Hypohidrotic Ectodermal Dysplasia: A Case Report
Krunal S. Soni1, Satabdi Saha1, Niharika1, Subrata Saha2
1Post Graduate Student, 2Professor,
Department of Pedodontics and Preventive Dentistry, Dr. R. Ahmed Dental College and Hospital, Kolkata, West Bengal.
Corresponding Author: Krunal S. Soni
Ectodermal dysplasia is a heterogenous group of hereditary disorders affecting development of two or more ectodermally derived anatomic structures. The Incidence of the syndrome is considered to be around 1 case per 100,000 live births. The two most common types of ectodermal dysplasias are hypohidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome), and hidrotic ectodermal dysplasia (Clouston syndrome). Hypohidrotic ectodermal dysplasia is usually inherited as X-linked recessive pattern and has a full expression in males, whereas females show little to no signs of the disorder. The triad of nail dystrophy, alopecia or hypotrichosis and palmoplantar hyperkeratosis is usually accompanied by a lack of sweat glands and a partial or complete absence of primary and/or permanent dentition. In this case report prosthetic rehabilitation of a young boy with Hypohidrotic ectodermal dysplasia is presented.
Key words: hypodontia, hypohidrotic ectodermal dysplasia, removable overdentures, prosthetic rehabilitation