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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).

ORPHA:2721

Classification level: Disorder
  • Synonym(s):
    • OODD
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Childhood
  • ICD-10: Q82.4
  • ICD-11: LD27.0Y
  • OMIM: 257980
  • UMLS: C0796093
  • MeSH: C537742
  • GARD: 4054
  • MedDRA: -

  A summary on this disease is available in Nederlands (2021) Deutsch (2007) Français (2007) Italiano (2007)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.