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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.
ORPHA:2721Classification level: Disorder
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.
Transmission is autosomal recessive.