Hypogonadotropic Hypogonadism-Frontoparietal Alopecia Syndrome

This syndrome is characterized by the association of hypogonadotropic hypogonadism and frontoparietal alopecia.

Epidemiology

It has been described in six members (four males and two females) of a consanguineous Lebanese family.

Clinical description

In addition to frontoparietal alopecia, the remaining scalp hair was also sparse, and axillary and pubic hair was absent.

Etiology

In contrast to the syndrome of primary hypergonadotropic hypogonadism and partial alopecia (see this term), streak or absent ovaries, hypoplastic uterus, and microcephaly were not reported in the affected female members of this Lebanese family.

Genetic counseling

Despite the family history of consanguinity, autosomal dominant inheritance with reduced penetrance was considered as the most likely mode of transmission.