A rare form of gynecomastia with hypogonadism. Similar to the Reifenstein Syndrome, but without hypospadias.
First described by Stanley Rosewater, an American endocrinologist, in 1965.
X-linked recessive or autosomal dominant.
It has been suggested that this condition is the mildest expression of incomplete male pseudohermaphroditism, type I. Masculinization by monthly administration of testosterone has been demonstrated.
Clinical features (gynecomastia with hypogonadism); biochemical (increased levels of testosterone and estrogen, but with low levels of luteinizing hormones); histology (decreased Leydig cells on testicular biopsy).
Gynecomastia, hypogonadism, sterility.
Endocrine dysfunction appears to be limited to the reproductive system. Psychological attention must be given to patients affected with this condition because of the embarrassment caused by the gynecomastia.
Other condition to be considered
et al: Androgen insensitivity syndrome. Lancet
SJ: Familial gynaecomastia. Ann Intern Med