A rare form of gynecomastia with hypogonadism. Similar
to the Reifenstein syndrome but without hypospadias.
First described by S. Rosewater in 1965.
X-linked or autosomal dominant.
Unclear; resistance to androgen action suggested.
Masculinization by monthly administration of testosterone has been
Clinical features (gynecomastia with hypogonadism);
biochemical (increased levels of testosterone and estrogen, but with low
levels of luteinizing hormones); histology (decreased Leydig cells on
Gynecomastia, hypogonadism, sterility.
Endocrine dysfunction appears to be
limited to the reproductive system. Psychological attention must be given to
patients affected with this conditions because of the embarrassment caused
by the gynecomastia.
Reifenstein Syndrome: Characterized by
male pseudohermaphroditism, which means that the individual has testes but
presents clinically with the secondary sexual characters both male and
female. The severity of androgen insensitivity determines the clinical
Rosewater S, Gwinup G, Hamwi SJ: Familial gynaecomastia. Ann Intern Med