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International journal of odontostomatology

On-line version ISSN 0718-381X

Abstract

ALVES, Nilton; OLIVEIRA, Reinaldo José de  and  DEANA, Naira Figueiredo. Hypohidrotic Ectodermal Dysplasia : a Syndrome of Interest to Dentistry. Int. J. Odontostomat. [online]. 2012, vol.6, n.1, pp.45-50. ISSN 0718-381X.  http://dx.doi.org/10.4067/S0718-381X2012000100006.

Hypohidrotic ectodermal dysplasia (HED) is a rare disease of genetic etiology. The most frequent form is of recessive linked to X-chromosome inheritance with affected male and female carriers. It can occur through autosomal mutations, of the gene EDA1 gene being responsible for the majority of the cases. It is characterized by the triad: hypohidrosis, oligodontia and hypotrichosis. We present two cases of patients with HED in which we observed characteristic signs of this syndrome: delicate skin, sparce hair, eyebrows and eyelashes, periorbital wrinkles, perioral and periorbital hyperpigmentation, prominent lips, in addition the patient in case 2 also present the depressed nasal bridge. We also found decreased salivary and lacrimal secretion and maxillary hypoplasia in both cases. At the oral examination in case 1 the upper right and left deciduous canines and lower right deciduous canine were present, and in case 2 the upper and lower (right and left) deciduous canines and two upper (one right and other left) permanent incisors were present with altered morphology, all of these dental elements were healthy. The early dental treatment of patients with HED, especially in the presence of oligodontia, as observed in our cases, is important not only to provide a better quality of life for these patients in the short term, but also an attempt to minimize the changes in facial growth to which these patients are subject.

Keywords : hypohidrotic ectodermal dysplasia; oligodontia; Christ-Siemens Touraine syndrome.

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