Hirsutism

Published on 21/03/2015 by admin

Filed under Pediatrics

Last modified 22/04/2025

Print this page

This article have been viewed 1136 times

Chapter 24 HIRSUTISM

Theodore X. O’Connell

General Discussion

The following discussion of hirsutism focuses on adolescent girls. For a discussion of hirsutism in infants or children, please see Chapter 35, Precocious Puberty.

Hirsutism is defined as the presence of excessive coarse terminal hair in a pattern not normal in females in areas such as the face, chest, or upper abdomen. This disorder is a sign of increased androgen action on hair follicles, which may result from increased levels of endogenous or exogenous androgens, or it may result from increased sensitivity of hair follicles to normal levels of circulating androgens. Hirsutism must be distinguished from hypertrichosis, which is the term used to describe the androgen-independent growth of hair that is vellus and does not follow the bodily distribution of the hair associated with male secondary sexual characteristics. Hypertrichosis may be familial or may be caused by medications or metabolic disorders.

Any patient who has either physical evidence of hirsutism or significant concern over excess hair growth should be evaluated. When evaluating hirsutism, it is important to determine whether hirsutism exists alone or whether virilization is also present. This distinction is important because virilization may reflect a serious underlying pathologic condition such as malignancy. Virilization manifests with a wide range of signs of androgen excess, such as acne, hirsutism, frontotemporal balding, amenorrhea, oligomenorrhea, deepening of the voice, and clitoromegaly.

The most common triggering factor for hirsutism is excess androgen production. Although androgens may come from an exogenous source, androgen excess is most commonly endogenous. The two primary sources of endogenous androgens are the adrenal glands and the ovaries. The most common cause of hyperandrogenism presenting in a teenage girl is polycystic ovary syndrome (PCOS). The differential diagnosis also includes late-onset congential adrenal hyperplasia, virilizing tumors, Cushing syndrome, hyperprolactinemia, acromegaly, medications, and abnormalities of androgen action or metabolism.

A common endocrine disorder, PCOS affects 3% to 10% of premenopausal women. It represents a group of disorders associated with increased androgen production from the ovaries, adrenal glands, or both. The clinical presentation typically includes one or several of the following features: hirsutism, obesity, oligomenorrhea, anovulation, and infertility. At the 1990 National Institutes of Health (NIH) consensus conference, experts attempted to identify the key features needed to diagnose PCOS, noting the following as definite criteria: hyperandrogenism, menstrual dysfunction, clinical evidence of hyperandrogenism, and the exclusion of congenital adrenal hyperplasia. Probable criteria included insulin resistance, perimenarchal onset, an elevated ratio of luteinizing hormone (LH) to follicle stimulating hormone (FSH), and polycystic ovaries by sonography.

Patients with PCOS must be differentiated from individuals with variants of nonclassic congenital adrenal hyperplasia (CAH). The most common form of CAH is late-onset 21-hydroxylase deficiency. The diagnosis of nonclassic CAH can be made definitively by the use of adrenocorticotropin hormone (ACTH)-stimulation testing or can be inferred by measurement of early morning 17-hydroxyprogesterone levels.

Suggested Work-up

Serum testosterone and DHEA sulfate (DHEA-S)	To evaluate for ovarian and adrenal tumors. Total testosterone levels >1.5 ng/mL or DHEA-S levels >700 μg/dL are suggestive of androgen-producing neoplasms.
Serum 17α-hydroxyprogesterone (17-OHP)	To evaluate for late-onset adrenalhyperplasia
	<200 ng/dL is unlikely to be 21-hydroxylase deficiency
	200-400 ng/dL indicates low likelihood 21- hydroxylase deficiency, but ACTH stimulation test recommended
	400-1000 ng/dL is suggestive of 21- hydroxylase deficiency and ACTH stimulation test is recommended
	>1000 ng/dL is diagnostic of 21- hydroxylase deficiency
Serum prolactin	To evaluate for pituitary tumors
Thyroid-stimulating hormone (TSH)	To evaluate for thyroid dysfunction
Fasting serum glucose	To evaluate for insulin resistance in patients suspected of having PCOS

Additional Work-up

ACTH stimulation test with measurement of 17-OHP at baseline and 60 minutes after administration of 0.25 mg cosyntropin	When CAH is suspected. In 21-hydroxylase deficiency, an increase of 17-OHP to more than 1500 ng/dL at 60 minutes is usually seen.
LH and FSH	May be useful in confirming the diagnosis of PCOS. Elevated LH level is suggestive of PCOS, particularly when the LH-FSH ratio exceeds 2.5.
24-hour urinary collection for free cortisol and creatinine levels	If Cushing syndrome is suspected
Dexamethasone suppression test (1 mg dexamethasone at 11 PM with 8 AM serum cortisol levels measured the next day)	If urinary free cortisol level is elevated. The morning cortisol level should be less than 5.0 μg/dL after the dexamethasone dosing.
Glucose tolerance test	In patients with suspected PCOS with elevated fasting serum glucose
Computed tomography (CT) of the abdomen and pelvis	To assess the adrenal glands and ovaries in patients whose history, physical examination, or laboratory evaluation suggest the presence of a virilizing tumor

1. Bailey-Pridham D.D., Sanfilippo J.S. Hirsutism in the adolescent female. Pediatr Clin North Am. 1989;36:581–599.

2. Bates G.W. Hirsutism and androgen excess in childhood and adolescence. Pediatr Clin North Am. 1981;28:513–530.

3. Gilchrist V.J., Hecht B.R. A practical approach to hirsutism. Am Fam Physician. 1995;52:1837–1846.

4. Gordon C.M. Menstrual disorders in adolescents: excess androgens and the polycystic ovary syndrome. Pediatr Clin North Am. 1999;46:519–543.

5. Hunter M.H., Carek P.J. Evaluation and treatment of women with hirsutism. Am Fam Physician. 2003;67:2565–2572.

6. Leung A.K., Robson W.L. Hirsutism. Int J Dermatol. 1993;32:773–777.

7. Miller W.L. Pathophysiology, genetics, and treatment of hyperandrogenism. Pediatr Clin North Am. 1997;44:375–395.

8. Plouffe L. Disorders of excessive hair growth in the adolescent. Obstet Gynecol Clin. 2000;27:79–99.

9. Redmond G.P., Bergfeld W.F. Diagnostic approach to adrogen disorders in women: acne, hirsutism, and alopecia. Cleve Clin J Med. 1990;57:423–427.

10. Rosenfield R.L. Hyperandrogenism in peripubertal girls. Pediatr Clin North Am. 1990;37:1333–1358.

11. Speroff L., Glass R.H., Kase N.G. Clinical Gynecologic Endocrinology and Infertility, 6th ed. Baltimore: Lippincott Williams & Wilkins; 1999. 529–556

Related

Instant Work-ups A Clinical Guide to Pediatrics