Steroid hormones in the adrenal venous effluents in idiopathic hirsutism under basal and stimulated conditions.

Steroid hormone concentrations in the peripheral blood and the adrenal veins were measured in the basal state and after ACTH stimulation in 5 patients with idiopathic hirsutism. The basal concentrations of the steroids in the adrenal veins of the patients with idiopathic hirsutism were not significantly different from a control group of 5 patients catheterized for investigation of pheochromocytoma. Following ACTH stimulation, the concentrations of the steroids in the adrenal veins were also not significantly different in the hirsute and the control groups except for the concentrations of DHA and DHAS which were higher in the patients with idiopathic hirsutism. 17-hydroxyprogesterone (17-OHP) concentrations after ACTH stimulation were lower in the hirsute group compared to the control population. It is concluded that patients with idiopathic hirsutism have a defect in the biosynthesis of cortisol proximal to the action of the 11 beta- and 21-hydroxylase enzymes, deficiencies of which have been previously considered to be the usual causes of hirsutism due to an adrenocortical abnormality. The lower 17-OHP concentrations in the hirsute group can be explained on the basis of deficiency of substrate for the action of the 17-hydroxylating enzyme, consequent to the postulated deficiency of 3 beta-hydroxysteroid dehydrogenase.