Odontomicronychial Dysplasia

Clinical Features

Pinheiro et al. (1996) described a combination of precocious eruption and shedding of deciduous teeth, precocious eruption of secondary teeth with short rhomboid roots, and short, thin, slow growing nails in a 7-year-old boy born of consanguineous parents of Portuguese ancestry. Four other members of the same family (3 males, 1 female), also born of consanguineous parents, reportedly had the same complex of abnormalities. Five affected persons had 11 normal sibs, and 2 affected persons had 8 normal children. Pinheiro et al. (1996) suggested that this condition, which they designated odontomicronychial dysplasia, is a unique form of ectodermal dysplasia.

Inheritance

Pinheiro et al. (1996) concluded that odontomicronychial dysplasia is an autosomal recessive disorder.