odontoonychodermal dysplasia

Epidemiology

rare

Pathology

ectodermal dysplasia

Genetics

• autosomal recessive
• associated with defects in WNT10A

Clinical manifestations

• presenting phenotype is dry hair, severe hypodontia, smooth tongue with marked reduction of fungiform & filiform papillae, onychodysplasia, keratoderma & hyperhidrosis of palms & soles, & hyperkeratosis of the skin

More general terms

• genetic syndrome (multisystem disorder)

Database

• OMIM: https://mirror.omim.org/entry/257980