This report describes a 17-yr-old female who developed acanthosis nigricans, hirsutism and amenorrhea concurrently at age 15. Plasma androstenedione (A) was extremely elevated ranging from 3500 to 5000 ng/100 ml. Plasma testosterone (T) and urinary 17-ketosteroids (17-KS) were also elevated .at 357 ng/100 ml and 32-42 mg/24 hr respectively. Plasma A and T were not reduced by dexamethasone (Dex), but human chorionic gonadotropin (hCG) increased plasma A to 23,500 ng/100 ml, plasma T to 3666 ng/100 ml and the 17-KS to 156 mg/24 hr. Ethinyl estradiol (EE) plus Dex reduced LH to a nondetectable level, A to 72 ng/100 ml and T to 10 ng/100 ml. ACTH did not increase A or T. The patient was treated for 7 months with 0.15 mg/day of EE and there was marked improvement of the acanthosis nigricans associated with correction of the hyperandrogenism. Because of menorrhagia, EE was discontinued and the high levels of A and T returned and the acanthosis nigricans worsened. Exploratory laparotomy was performed and bilateral polycystic ovarian disease and a stromal luteoma or lipoid cell tumor of the right ovary was found. The acanthosis nigricans improved following bilateral ovarian wedge resection but soon worsened. The patient was then placed on a combination-type oral contraceptive containing norethindrone 2 mg and mestranol 0.1 mg (Ortho-Novum 2 mg) and plasma A and T were again suppressed. The acanthosis nigricans has almost completely disappeared on this suppressive therapy. It is concluded that the steroid secretion by the stromal luteoma was not autonomous but gonadotropin dependent. Pituitaryovarian suppression corrected the hyperandrogenism and was associated with improvement in the acanthosis nigricans.
ASJC Scopus subject areas
- Endocrinology, Diabetes and Metabolism
- Clinical Biochemistry
- Biochemistry, medical