Hereditary enamel hypoplasia

An extensive family with a total of forty–one individuals who are either affected or reputed to be affected with hereditary hypoplasia of the enamel is presented. The distribution of patients, which spans four generations in this pedigree, appears to be consistent with X–linked dominant inheritance of the disorder. Considerable variability in the severity of affection was observed among both male and female patients. Whereas in the case of the latter, random X–chromosome inactivation may provide an explanation, in the case of the former another mechanism may have to be sought.