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Odonto-onycho-dermal dysplasia

Synonym(s) OODD
Prevalence <1 / 1 000 000
Inheritance Autosomal recessive
Age of onset Childhood
  • Q82.4
  • C1275074
  • C537742
MedDRA -


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.


Less than 15 cases have been described so far.


Homozygous mutations have been described in the WNT10A gene in six patients born to three consanguineous families.

Genetic counseling

Transmission is autosomal recessive.

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