Ectodermal Dysplasia: Series of 8 Cases - Abstract
Introduction: Hypohidrotic ectodermal dysplasia is a genetic disease of ectoderm development characterized by malformations of ectodermal structures such as the skin, hair, teeth and sweat glands.
Material and methods: We report the cases of 8 children.
Results: The mode of diagnosis, the clinical signs and the therapeutic option are detailed.
Discussion: Hypohidrotic ectodermal dysplasia is characterized by the triad of manifestations including sparse hair (atrichosis / hypotrichosis), missing teeth (anodontia / hypodontia) or abnormal (for example conical) and reduced or absent sweating due to the absence of sweat glands (anhidrosis / hypohidrosis) leading to heat intolerance. The treatment is based on the eviction of uncontrolled exposure to heat with an early dental treatment which aims to restore dental function and improve the appearance of teeth as well as orthodontic treatment. Most patients have normal life expectancy with early diagnosis and appropriate management.