Orphanet: Odonto onycho dermal dysplasia

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Odonto-onycho-dermal dysplasia

Disease definition

A rare, genetic, ectodermal dysplasia syndrome characterized by dental abnormalities (primarily agenesis of the permanent and deciduous teeth with cone-shaped incisors and canines), onychodysplasia, palmoplantar hyperkeratosis, dry skin and, more variably, hypotrichosis, and sweat gland dysfunction (hyper- or hypohidrosis).


Classification level: Disorder
  • Synonym(s):
    • OODD
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Childhood
  • ICD-10: Q82.4
  • OMIM: 257980
  • UMLS: C0796093  C1275074
  • MeSH: C537742
  • GARD: 4054
  • MedDRA: -
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