Trichodental Dysplasia

Salinas and Spector (1980) reported 2 pedigrees in which an association of hypodontia and abnormal hair was found in 10 persons. Autosomal dominant inheritance of the disorder was confirmed in 2 other families (Kersey, 1987; Eteson and Clark, 1989). Missing teeth, peg-shaped incisors, and shell teeth were the most common dental abnormalities. The hair in most patients was fine, sparse, dull, and slow growing. There was considerable intra- and interfamilial variability. Giannotti et al. (1995) described a sporadic case of trichodental dysplasia in an Italian girl who also had mild microcephaly and mental retardation.