Hereditary ectodermal dysplasia of the anhidrotic type associated with primary hypogonadism

Abstract Two cases of hereditary ectodermal dysplasia of the anhidrotic type are described. There was associated primary hypogonadism. The mode of inheritance of this syndrome is reviewed. It appears that a sex-linked recessive gene which is sometimes incompletely recessive would account for most of the published cases. The pedigree of our patients is compatible with this type of transmission but also could be explained by a dominant gene with incomplete penetrance or by a non-sex-linked recessive gene.