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hirsutism

An androgen-dependent condition in women characterized by excessive growth of body hair following a male distribution pattern. Etiology: 1) idiopathic 2) polycystic ovary disease (50-70%) [3] 3) hyperandrogenism, insulin resistance & acanthosis nigricans (HAIR AN) 4) adrenal hyperplasia 5) 21-hydroxylase deficiency 6) 11-hydroxylase deficiency 7) Cushing's syndrome 8) adrenal neoplasm a) adrenal adenoma b) adrenal carcinoma 9) ovarian neoplasm 10) prolactinoma [3] 11) arrhenoblastoma (Sertoli-Leydig cell tumor) 12) gynandroblastoma 13) thecoma 14) Brenner tumor 15) lipid cell tumor 16) gonadoblastoma 17) pharmacologic a) testosterone containing topical agents b) anabolic steroids: DHEA c) danazol d) progesterone-dominant oral contraceptives e) high-dose glucocorticoids f) acetazolamide [3] g) corticotropin [3] Clinical manifestations: - excessive growth of body hair following a male distribution pattern - terminal hairs on the abdominal midline below the umbilicus - deepening of voice - acne - clitoromegaly - decreased breast size - male balding pattern [3] * images [6,7] Laboratory: 1) serum DHEA-sulfate (DHEAS) [0.4-3.2 ug/mL] - values > 8 ug/mL diagnostic for androgen-producing adrenal carcinoma [2] 2) serum testosterone [0.2-0.7 ng/mL] a) if > 200 ng/mL, suspect ovarian tumor b) if < 200 ng/mL, suspect polycystic ovary disease or other cause c) normal serum DHEA (serum DHEAS) with high serum testoterone suggests ovarian source of androgen excess [3] 3) screening tests for Cushing's syndrome a) 24 hour urinary free cortisol b) overnight dexamethasone suppression test 4) serum androstenedione [0.5-2.5 ng/dL] 5) serum luteinizing hormone (LH) [5-25 mIU/mL] 6) serum follicle-stimulating hormone [5-20 mIU/mL] 7) serum prolactin [2-15 ng/mL] 8) 17-hydroxyprogesterone in serum* [3] 9) CA-125 in serum [3] * CT of adrenals & ovaries is 1st line [10] Radiology: - transvaginal ultrasound to examine ovaries [3] - 1st test when serum testosterone > 150 ng/dL - computed tomography to visualize both adrenals & ovaries - 1st test when serum DHEA-sulfate > 700 ug/dL [3] - 1st test after onset of virilization in women [10] Differential diagnosis: - in contrast to hypertrichosis, the excess hair in hirsutism is terminal hair; there is no increase in vellus hair Management: 1) cosmetic depilation 2) bleaching of hairs 3) medical (based upon reducing/antagonizing androgens) a) oral contraceptives (first line) [3] 1] idiopathic hirsutism 2] polycystic ovary disease b) spironolactone (androgen antagonist) is added only when oral contraceptive is insufficient [3] - teratogenic, do not use without contraception c) eflornithine d) finasteride [3] 4) obesity control 5) glucocorticoids for management of late onset congenital adrenal hyperplasia

Related

Cushing's syndrome; hypercortisolism; hyperadrenocorticism; pluriglandular syndrome Stein-Leventhal (polycystic ovary) syndrome

General

hairiness chronic endocrine disease

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 619-21
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 243-46
  3. Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18. American College of Physicians, Philadelphia 2012, 2015, 2018.
  4. Martin KA, Chang RJ, Ehrmann DA, Ibanez L et al Evaluation and treatment of hirsutism in premenopausal women: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2008 Apr;93(4):1105-20 PMID: 18252793
  5. Curran DR, Moore C, Huber T. Clinical inquiries. What is the best approach to the evaluation of hirsutism? J Fam Pract. 2005 May;54(5):465-7 PMID: 15865908
  6. Griffing GT, Rivlin ME (images) Medscape: Hirsutism http://emedicine.medscape.com/article/121038-overview
  7. DermNet NZ. Hirsutism (images) http://dermnetnz.org/hair-nails-sweat/hirsutism.html
  8. Mimoto MS, Oyler JL, Davis AM Evaluation and Treatment of Hirsutism in Premenopausal Women. JAMA. Published online March 9, 2018 PMID: 29522641 https://jamanetwork.com/journals/jama/fullarticle/2674903
  9. Mihailidis J, Dermesropian R, Taxel P Endocrine evaluation of hirsutism. Int J Womens Dermatol. 2017 Feb 16;3(1 Suppl):S6-S10. Review. PMID: 28492032 Free PMC Article
  10. NEJM Knowledge+ Endocrinology