An unusual case of male pseudohermaphroditism.

In vivo and in vitro studies were conducted on a male pseudohermaphrodite. Levels of gonadal steroids in peripheral blood suggested low activity of steroidogenesis which was consistent with the high levels of gonadotropins found. In vitro incubation studies with testosterone, human chorionic gonadotropin (hCG) and follicle-stimulating hormone (FSH) indicated that both gonadotropins increased the endogenous accumulation of testosterone (P less than 0.05) and the rate of conversion of testosterone to androstenedione (P less than 0.025). Estradiol accumulation was increased only in the presence of hCG. These data suggest that the aromatase and 17 beta-hydroxysteroid dehydrogenase enzyme systems in the gonads of this patient may be influenced by gonadotropins.