Do you know this syndrome? Clouston syndrome
AUTOR(ES)
Sanches, Sarah, Rebellato, Priscila Regina Orso, Fabre, Andréa Buosi, Campos, Giovana Liz Marioto de
FONTE
An. Bras. Dermatol.
DATA DE PUBLICAÇÃO
2017-06
RESUMO
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.
