Odonto-ungueal dysplasia: an apparently new autosomal dominant ectodermal dysplasia

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Odonto-ungueal dysplasia: an apparently new autosomal dominant ectodermal dysplasia

Marta Pinheiro; Newton Freire-Maia

Departamento de Genética, Universidade Federal do Paraná, Caixa Postal 19071, 81531-990 Curitiba, PR, Brasil. Send correspondence to M.P.

ABSTRACT

We describe 27 subjects (11 women) from five generations of a family with an apparently hitherto undescribed ectodermal dysplasia. All of them presented dental and/or nail alterations only. A genetic analysis of the family suggests an autosomal dominant gene. Differential diagnosis considered eight conditions belonging to the same odonto-onychic (2-3) subgroup, as well as Fried's tooth and nail syndrome and hypodontia and nail dysgenesis (both in 1-2-3 subgroup).

Keywords: odonto-ungueal dysplasia; autosomal dominant; ectodermal dysplasia.

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Publication Dates

• Publication in this collection
  26 Oct 2006
• Date of issue
  1996