Hirsutism, development of terminal body hair in women in unexpected areas, may result from different etiologies; 4.3% being nonclassical congenital adrenal hyperplasia (NCCAH) and acromegaly being one of the rarests. We here present a severe hirsutism case with acromegaly and NCCAH.
Case: A 30-year-old woman with headache and visual field abnormalities was consultated by our clinic about her cranial imaging revealing a macroadenoma in pituitary localization. She had had secondary amenorrea, infertility and progressive hirsutism for 3 years and had been on contraceptives irregularly and on regular cabergoline treatment for 2 years. On physical examination; blood pressure: 140/100 mmHg, pulse: 86/min, body mass index: 33.057 kg/m2. She had distinct hirsutism with a Ferriman-Gallwey score of 31 (n<8). She had no acromegaloid or cushingoid features. On hormonal evaluation, prolaktin: <0.50 mg/ml (1.925), GH: 5.22 ng/ml (0.065), IGF1: 422 ng/ml (69358), Total testosterone: 184 ng/dl (1580), androstenedione: 4.58 ng/ml (0.33.5), 17-OH progesterone: 1 ng/ml (<2). Cortisol was supressed below 1.8 μg/dl in low dose dexamethasone supression test, but nadir GH level in oral glucose tolerance test was 1.56. There was a 14 mm macroadenoma in the pituitary and bilateral hemianopsia on visual field examination. She underwent transsphenoidal surgery with a presumptive diagnosis of acromegaly. Pathological specimen was 60% GH positive. After surgery acromegaly was under control but her symptoms persisted. The re-evaluation of the hormones revealed 17-OH progesterone: 3.2 ng/dl, which rised above 10 ng/dl with ACTH stimulation test. Thus, she was accepted as NCCAH. She is now on prednisolone 5 mg/day therapy and has started to have regular menses.
Conclusion: Hirsutism is a relatively uncommon but reported finding in acromegalics. We detected concomitant acromegaly and NCCAH in our patient. While evaluating for hirsutism, one should rule out all the possible underlying etiologies, especially endocrinopathies even though a distinct reason has already been detected.
30 Apr - 04 May 2011
European Society of Endocrinology