GONADAL DYSGENESIS WITH ANDROGENIC MANIFESTATIONS IN THE TALL EUNUCHOID FEMALE*†

Abstract

A syndrome is reported which is characterized by rudimentary ovaries, infantile uterus, complete lack of breast development, moderate amounts of sexual and axillary hair, tall stature, clitoridal hypertrophy, a female sex chromatin pattern in the skin, normal to slightly elevated urinary follicle stimulating hormone (FSH) low urinary 17-ketosteroids, and clinical manifestations of hypoestrinism. This case may furnish a link in the relationship between the ovarian agenesis of Albright et al and the ovarian dysgenesis with masculine manifestations described by Gordan et al. The syndrome should be considered a variant of the syndrome of ovarian agenesis and perhaps a subform of hermaphrodism, and should be differentiated from the various types of heterosexual development.