Hirsutism - excess female facial and/or body hair Hyperandrogenism in women - excess male hormones in women
What is hirsutism?
- Excess facial and body hair growth in women.
What is hyperandrogenism?
- Increased levels of male hormone production in women.
Diagnostic classification of hyperandrogenism
These are the causes of hyperandrogenism and hirsutism in women:
- Chronic anovulation - i.e. polycystic ovarian syndrome
- Idiopathic hirsutism - excess hair growth with normal menstrual cycles and normal androgen (male hormone) levels
- Late onset congenital adrenal hyperplasia - an inherited disorder of hormone metabolism that causes increased hair growth in women after puberty
- Ovarian tumors - a rare cause of hirsutism
- Adrenal causes - even more rare
- Pregnancy related - rare, but interesting, e.g. luteoma of pregnancy
- Drugs - some drugs can cause excess hair growth in women
Evaluation of hirsute women
Women with hirsutism have an increased production rate of testosterone and androstenedione.
The most commonly seen clinical problem is the typical story of irregular menstrual cycles (anovulation), onset of hirsutism in the teens or early 20s, and gradually worsening excess hair growth. These are the findings associated with a condition known as polycystic ovarian syndrome.
Initial testing for hirsute women
- Total testosterone
- Dehydroepiandrosterone sulfate (DHEAS)
If the woman is anovulatory, she also should have a prolactin, T4 and TSH.
A breast exam should be done to check for galactorrhea. If the woman has long-standing anovulation, an endometrial biopsy may be indicated.
Late onset CAH, Congenital Adrenal Hyperplasia
- 21-hydroxylase deficiency- relatively common
- 3-beta-hydroxysteroid dehydrogenase deficiency - subtle, accurate diagnosis not essential
- 11-beta-hydroxylase deficiency - rare
- Late onset 21-hydroxylase deficiency
- Autosomal recessive transmission - homozygous for the mild allele, or 1 mild and 1 severe allele.
- Late onset CAH affects about 1-5% of hyperandrogenic women.
- The screening test for this condition is a morning 17-hydroxyprogesterone.
- The 17-OHP result should be less than 200 ng/dl. A level of 200-800 ng/dl requires ACTH stimulation testing to clarify the situation.
- A level of over 800 ng/dl is diagnostic for late onset 21-hydroxylase deficiency.
Testosterone (T) levels
- 70% of women with anovulation and hirsutism have elevated plasma testosterone levels (> 80 ng/dl)
- Sex hormone binding globulin levels are decreased by androgen and insulin, so total testosterone can be normal while the active, unbound testosterone is elevated.
- If the total testosterone is over 200 ng/dl, a tumor should be considered as a possibility.
- Tumors causing hirsutism are rare and other causes are common.
- A tumor workup is indicated in most cases if total testosterone is over 200 ng/dl (100 ng/dl if the woman is menopausal).
- Indicated in most cases if DHEAS is over 700 ug/dl (400 ug/dl if menopausal).
Treatment of hyperandrogenism
This aldosterone-antagonist diuretic inhibits synthesis of male hormones, competes for androgen receptors in hair follicles, and directly inhibits 5-a-reductase. This is the enzyme that converts testosterone to the active hormone dihydrotestosterone.
Dose to use is 100-200 mg/day. There are usually few side effects. But sometimes women complain of increased urination in the first few days of use, fatigue, or problems with their menstrual periods. Spironolactone works best when used in conjunction with oral contraceptives. This also controls the menstrual cycle to avoid problems with dysfunctional bleeding.
A nonsteroidal antiandrogen that can be used at a dose of 250 mg three times daily for hirsutism. Its use seems to be relatively free of side effects. However, it should be used in conjunction with a method of contraception.