Hypohidrotic ectodermal dysplasia. Review of ENT manifestations on purpose of two cases

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René Sepúlveda S.
Daniel Muñoz S.
Felipe Cardemil M.
Maritza Rahal E.

Keywords

Hypohidrotic ectodermal dysplasia, ozena, epistaxis

Abstract

Hypohidrotic ectodermal dysplasia is an uncommon, hereditary disease, clinically characterized by the triad of hypohidrosis, dental deficiencies and lack of hair growth. In addition, it affects other skin appendages such as hair follicles, nails and teeth and even structures such as the mucous glands of the upper aerodigestive tract causing signs and also symptoms and pathologies of interest for the otolaryngologist. Two cases are presented, an adult and a child one,an in addition to literature review regarding the manifestations of hypohidrotic ectodermal dysplasia in head and neck.

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