odonto-onycho-dermal dysplasia

odonto-onycho-dermal dysplasia

A rare autosomal recessive ectodermal dysplasia (OMIM:257980) characterised by dry hair, severe hypodontia, smooth tongue with marked reduction of papillae, onychodysplasia, keratoderma and hyperhidrosis of palms and soles, and cutaneous hyperkeratosis.

Molecular pathology
Caused by defects in WNT10A cause odonto-onycho-dermal dysplasia.