Böök syndrome (PHC)
The features of Böök syndrome include premolar agenesis, hyperhidrosis of the hands and feet, and early graying of the hair (canities prematura). Early, diffuse whitening of the hair may appear even in childhood. Hypodontia in the Böök syndrome affects the premolar region, with one or more premolars missing. The syndrome has an autosomal dominant inheritance with high or complete penetrance; the gene defect is unknown (Gorlin et al, 1990; Böök, 1950).
Text adapted from Sirpa Arte: Phenotypic and genotypic features of familial hypodontia. Dissertation, Institute of Dentistry, University of Helsinki, Finland, 2001.
Text last edited 09.10.2003 by P.N. , page last created 10.12.2004 by P.N.