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Hypohidrotic ectodermal dysplasia: dental, clinical, genetic and dermatoglyphic findings of three cases

  • B. Kargül1,*,
  • T. Alcan2
  • U. Kabalay1
  • M. Atasu3

1Department of Pediatric Dentistry, Dental School, Marmara University, İstanbul, Turkey

2Department of Orthodontics, Dental School, Marmara University, İstanbul, Turkey

3Department of Genetics, Dental School, Marmara University, İstanbul, Turkey

DOI: 10.17796/jcpd.26.1.g156r02u66w30729 Vol.26,Issue 1,January 2002 pp.5-12

Published: 01 January 2002

*Corresponding Author(s): B. Kargül E-mail: b.kargul.ped@marun.edu.tr

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Abstract

Patients with hypohidrotic ectodermal dysplasia (HED) are characterized by the clinical manifestations of hypodontia, hypohidrosis, hypotrichosis and a highly characteristic facial physiognomy.This disorder is inherited as an X- linked trait. This report presents three cases with HED in which the clinical evalua-tion (intraoral and radiological), genetic findings and SEM examination of hair. Boys 6 to 14 year old and a 11 year old girl were referred to the Marmara University, Faculty of Dentistry, complaining of oligodontia in the maxillary and mandibular arches and delay in eruption of other teeth. Peg-shaped teeth have been observed. The dermatoglyphs of the patients were striking. SEM exmimation of hair demostrated a distinctly abnormal longitudinal groving along the entire length of each hair and a desqua-mation of the surface cuticles. The treatment was planned in a multidisciplinary odontological group involving pediatric dentistry, orthodontics, prosthodontics and oral surgery and maxillofacial radiology of future dental habilitation. A specially designed overdenture, a removable prosthesis and osseointe-grated implants were constructed. Periodic recall visits were advised, to monitor the dentures and implants during periods of growth and development, and eruption of the permanent teeth.


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B. Kargül,T. Alcan,U. Kabalay,M. Atasu. Hypohidrotic ectodermal dysplasia: dental, clinical, genetic and dermatoglyphic findings of three cases. Journal of Clinical Pediatric Dentistry. 2002. 26(1);5-12.

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https://www.jocpd.com/articles/10.17796/jcpd.26.1.g156r02u66w30729

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