Orphanet: Dysplasie, odonto onycho dermale

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Odonto-onycho-dermal dysplasia

Disease definition

Odonto-onycho-dermal dysplasia is a form of ectodermal dysplasia characterised by hyperkeratosis and hyperhidrosis of the palms and soles, atrophic malar patches, hypodontia, conical teeth, onychodysplasia, and dry and sparse hair.


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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