| Literature DB >> 29186264 |
Sarah Sanches1, Priscila Regina Orso Rebellato1, Andréa Buosi Fabre1, Giovana Liz Marioto de Campos1.
Abstract
Ectodermal dysplasias are conditions that present primary defects in two or more tissues of ectodermal origin and can be classified as hypohidrotic and hidrotic. Hidrotic ectodermal dysplasia or Clouston syndrome is an autosomal dominant genodermatosis and appears as a triad of clinical findings: palmoplantar keratoderma, nail dystrophy, and hypotrichosis. The hair is sparse and brittle. The nails become thickened and dystrophic, which is an essential characteristic of the syndrome. The diagnosis is made based on clinical findings. This study reports a case of a patient who began with changes in hair, nails and palmoplantar keratoderma in early childhood.Entities:
Mesh:
Year: 2017 PMID: 29186264 PMCID: PMC5514592 DOI: 10.1590/abd1806-4841.20175716
Source DB: PubMed Journal: An Bras Dermatol ISSN: 0365-0596 Impact factor: 1.896