title: [Hypohidrotic ectodermal dysplasias].
abstract: Ectodermal dysplasias form a heterogeneous group of hereditary diseases associating dysplastic abnormalities of four tissues which derive from the ectoderm. The frequency of these congenital diseases is estimated at 7 out of 100,000 newborns. More than 150 different syndromes have been described. Solomon and Keuer, then Freire Maya and Pinheiro established a classification of these diseases dividing them in 6 groups depending on the presence of the four main clinical disorders: trichodysplasia, onychodysplasia, hypodontia and hypohidrosis. We focus on the fundamental clinical features of the hypohidrotic ectodermal dysplasias and propose an overview of the most frequent forms based on a review of the literature.
authors.affiliation: Service de Dermatologie, CHU, Hôpital Nord, 42055 Saint-Etienne Cedex 2, France.
authors.forename: I
authors.initials: I
authors.lastname: Plottova-Puech
authors.forename: F
authors.initials: F
authors.lastname: Cambazard
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D004476:Ectodermal Dysplasia / Q000145:classification / Q000150:complications / Q000473:pathology*; D006801:Humans; D007007:Hypohidrosis / Q000209:etiology*; D015994:Incidence; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000453:epidemiology / Q000473:pathology; D012720:Severity of Illness Index