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Case Reports
. 2017 May-Jun;92(3):417-418.
doi: 10.1590/abd1806-4841.20175716.

Do you know this syndrome? Clouston syndrome

Sarah Sanches  1 , Priscila Regina Orso Rebellato  1 , Andréa Buosi Fabre  1 , Giovana Liz Marioto de Campos  1
Affiliations
Case Reports

Do you know this syndrome? Clouston syndrome

Sarah Sanches et al. An Bras Dermatol. 2017 May-Jun.

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.

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Conflict of interest statement

Conflict of interest: None

Figures

Figure 1
Figure 1
A: Dull and brittle dry hair. B: Plantar keratoderma. C: Nail plate dystrophy
Figure 2
Figure 2
Trichogram: A: Presence of irregular helical twist. B: Helical twist detail
See this image and copyright information in PMC

References

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