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Displasia ectodérmica hipoidrótica e suas implicações na cavidade oral
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O presente trabalho consta de uma revisão bibliográfica com o objetivo de estabelecer uma correlação entre a Displasia Ectodérmica Hipoidrótica e suas implicações diretas na cavidade oral.
A Displasia Ectodérmica é uma doença genética associada ao cromossoma X que engloba um conjunto de síndromes hereditárias. Este grupo de patologias envolve defeitos, com origem na ectoderme, de um ou mais tecidos durante a embriogénese, como os pelos, unhas, dentes e pele. A displasia ectodérmica hipoidrótica também conhecida como Síndrome de Christ-Siemens-Touraine é o fenótipo mais comum da doença, que se apresenta como uma malformação congénita caracterizada por hipotricose com diminuição ou ausência de pelos, hipodontia ou anodontia total associada com erupção atrasada dos dentes permanentes e redução da transpiração (hipoidrose).
The present work concerns a bibliographic review with the main objective of performing the correlation between hypohydrotic ectodermal dysplasia and its direct implications in the oral cavity. Ectodermal dysplasia is a genetic disease associated with the X chromosome, that encompasses a set of hereditary syndromes. This group of pathologies involve defects, with origin on the ectoderm, in one or more tissues during embryogenesis, such as the hair, nails, teeth and skin. Hypohydrotic ectodermal dysplasia also known as Christ-Siemens-Touraine Syndrome is the most common phenotype of the disease, which presents as a congenital malformation characterized by hypotrichosis with decreased or absence of hair, hypodontia or total anodontia associated with delayed eruption of permanent teeth and reduction of sweating (hypohidrosis).
The present work concerns a bibliographic review with the main objective of performing the correlation between hypohydrotic ectodermal dysplasia and its direct implications in the oral cavity. Ectodermal dysplasia is a genetic disease associated with the X chromosome, that encompasses a set of hereditary syndromes. This group of pathologies involve defects, with origin on the ectoderm, in one or more tissues during embryogenesis, such as the hair, nails, teeth and skin. Hypohydrotic ectodermal dysplasia also known as Christ-Siemens-Touraine Syndrome is the most common phenotype of the disease, which presents as a congenital malformation characterized by hypotrichosis with decreased or absence of hair, hypodontia or total anodontia associated with delayed eruption of permanent teeth and reduction of sweating (hypohidrosis).
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Displasia ectodérmica Causas genéticas Sinais Sintomas Ectodermal dysplasia Genetic causes Signs Symptoms