Abstract
A syndrome of scanty, fine, curled hair, thin dysplastic nails, taurodontic molars, hypoplastic-hypomature enamel, dysplasia of dentin, and hypohidrosis segregating as an autosomal dominant trait is described in a Japanese family. The patients received repeated doses of tetracycline during odontogenesis which fortuitously assisted in interpreting the pathogenesis of the dentin defect. The condition was previously described as familial congenital ectodermal dysplasia by Westerholm 1 and as odontogenesis imperfecta by Siirilä and Heikinheimo. 2 It is suggested that the name trichoonychodental (TOD) syndrome is a better descriptive term for the condition.
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