Hirsuitism - Clinicals, Diagnosis, and Management

Sexual and Reproductive Health

Clinicals - History

Fact Explanation
Excess hair Increased growth of terminal hair in women mainly on the chin, upper lip, breasts, upper back, and abdomen (in an androgen-dependent male distribution). Excess hair
Increased growth of terminal hair in women mainly on the chin, upper lip, breasts, upper back, and abdomen (in an androgen-dependent male distribution).
Age of onset Idiopathic hirsuitism is seen to occur shortly after puberty with slow progression. In premenopausal and menopausal women, hirsuitism occurs due to a decline in the secretion of ovarian estrogen with continuous androgen production. PCOS occurs in women of reproductive age. Patients presenting with premature pubarche and hirsutism in the prepubertal year can be suspected with nonclassical congenital adrenal hyperplasia which is typically caused by a deficiency of 21-hydroxylase. Age of onset
Idiopathic hirsuitism is seen to occur shortly after puberty with slow progression. In premenopausal and menopausal women, hirsuitism occurs due to a decline in the secretion of ovarian estrogen with continuous androgen production. PCOS occurs in women of reproductive age. Patients presenting with premature pubarche and hirsutism in the prepubertal year can be suspected with nonclassical congenital adrenal hyperplasia which is typically caused by a deficiency of 21-hydroxylase.
Gradual / sudden progression of hair growth Determining the progression of hair growth tells us whether the cause of hirsuitism is a benign condition (gradual progression) or malignant condition (sudden). Benign causes may include thyroid dysfunction, hyperprolactinemia, PCOS, CAH and anovulation. Malignant causes include ovarian and adrenal tumors. Gradual / sudden progression of hair growth
Determining the progression of hair growth tells us whether the cause of hirsuitism is a benign condition (gradual progression) or malignant condition (sudden). Benign causes may include thyroid dysfunction, hyperprolactinemia, PCOS, CAH and anovulation. Malignant causes include ovarian and adrenal tumors.
Menstrual and reproductive history Determine any history of infertility. Primary amenorrhea can indicate nonclassical congenital adrenal hyperplasia. Presence of irregular menses may indicate benign causes of hirsuitism. Menstrual and reproductive history
Determine any history of infertility. Primary amenorrhea can indicate nonclassical congenital adrenal hyperplasia. Presence of irregular menses may indicate benign causes of hirsuitism.
Family history Hirsuitism is manifested in patients with a family history of conditions such as polycystic ovary syndrome (PCOS), congenital nonclassical adrenal hyperplasia, HAIR-AN syndrome and conditions causing metabolic and cardiovascular dysfunction. Family history
Hirsuitism is manifested in patients with a family history of conditions such as polycystic ovary syndrome (PCOS), congenital nonclassical adrenal hyperplasia, HAIR-AN syndrome and conditions causing metabolic and cardiovascular dysfunction.
Drugs Use of pharmacologic drugs such as OCs, danazol, testosterone, anabolic steriods , metoclopramide, methildopa, phenothaizines, reserprine or valproic acid (Depakote) prior to the onset are known to elicit hirsuitism. Intake of anabolic steroids by female atheletes and body builders may experience hirsuitism and signs of virilzation. Drugs
Use of pharmacologic drugs such as OCs, danazol, testosterone, anabolic steriods , metoclopramide, methildopa, phenothaizines, reserprine or valproic acid (Depakote) prior to the onset are known to elicit hirsuitism. Intake of anabolic steroids by female atheletes and body builders may experience hirsuitism and signs of virilzation.
Flow of milk from breast Expressible or spontaneous galactorrhea indicates hyperprolactenemia. Flow of milk from breast
Expressible or spontaneous galactorrhea indicates hyperprolactenemia.
Mood and sleep disturbances, fragile skin and weight gain Indicates Cushing syndrome. Mood and sleep disturbances, fragile skin and weight gain
Indicates Cushing syndrome.

Clinicals - Examination

Fact Explanation
Excess hair Examine whether the excess hair growth in sex-specific areas of the body (male distribution pattern) or if there is a generalized increase in growth of hair on over the body which would indicate hypertrichosis (not caused by androgen excess). Excess hair
Examine whether the excess hair growth in sex-specific areas of the body (male distribution pattern) or if there is a generalized increase in growth of hair on over the body which would indicate hypertrichosis (not caused by androgen excess).
Acne, acanthosis nigricans, patterned hair loss, and seborrhea These are cutaneous signs of hyperandrogenism and acanthosis nigricans is a sign of insulin resistance. Acne, acanthosis nigricans, patterned hair loss, and seborrhea
These are cutaneous signs of hyperandrogenism and acanthosis nigricans is a sign of insulin resistance.
Swelling of the clitoris, deepening of the voice, acne, excessive facial and body hair Signs of virilization. Swelling of the clitoris, deepening of the voice, acne, excessive facial and body hair
Signs of virilization.
Striae, moon facies, fat redistribution, fragile skin, supraclavicular fat pad and proximal myopathy Indicates Cushing syndrome. Striae, moon facies, fat redistribution, fragile skin, supraclavicular fat pad and proximal myopathy
Indicates Cushing syndrome.
Textural skin changes, goiter, and hair loss Indicates thyroid disease. Textural skin changes, goiter, and hair loss
Indicates thyroid disease.
Expressible or spontaneous galactorrhea Indicates hyperprolactinemia. Expressible or spontaneous galactorrhea
Indicates hyperprolactinemia.
Coarse facies and enlarged hands and feet Indicates Acromegaly. Coarse facies and enlarged hands and feet
Indicates Acromegaly.
Visual field defect Suggests a pituitary adenoma. Visual field defect
Suggests a pituitary adenoma.
Palpable abdominal or pelvic mass Suggests an adrenal or ovarian mass. Palpable abdominal or pelvic mass
Suggests an adrenal or ovarian mass.

Investigations - Diagnosis

Fact Explanation
Ferriman-Gallwey score Determine whether the patient's hirsuitism is mild, moderate or severe by visually scoring the body and facial terminal hair growth in specified body areas by the Ferriman-Gallwey tool. Scores 8-15 means mild hirsutism and >15 means moderate to severe hirsutism. Ferriman-Gallwey score
Determine whether the patient's hirsuitism is mild, moderate or severe by visually scoring the body and facial terminal hair growth in specified body areas by the Ferriman-Gallwey tool. Scores 8-15 means mild hirsutism and >15 means moderate to severe hirsutism.
Luteinizing hormone (LH) /follicle-stimulating hormone (FSH)/ estradiol To evaluate infertility and ovulatory dysfunction. An increased LH:FSH ratio (> 3) is a common finding in PCOS. Luteinizing hormone (LH) /follicle-stimulating hormone (FSH)/ estradiol
To evaluate infertility and ovulatory dysfunction. An increased LH:FSH ratio (> 3) is a common finding in PCOS.
Metabolic evaluation Indicated if PCOS is suspected. Measure plasma glucose levels, waist circumference and body mass index, complete lipid profile, and blood pressure to evaluate the patient’s risk of metabolic and cardiovascular dysfunction. Metabolic evaluation
Indicated if PCOS is suspected. Measure plasma glucose levels, waist circumference and body mass index, complete lipid profile, and blood pressure to evaluate the patient’s risk of metabolic and cardiovascular dysfunction.
Thyroid function tests Indicated in patients with hirsuitism ,irregular menses and signs of thyroid disease. Measure thyroid-stimulating hormone (TSH) levels, free thyroxine, and thyroid peroxidase antibodies. Abnormal results indicate thyroid dysfunction. If results are normal, consider CAH, PCOS and anovulation. Thyroid function tests
Indicated in patients with hirsuitism ,irregular menses and signs of thyroid disease. Measure thyroid-stimulating hormone (TSH) levels, free thyroxine, and thyroid peroxidase antibodies. Abnormal results indicate thyroid dysfunction. If results are normal, consider CAH, PCOS and anovulation.
Prolactin Indicated in patients with hirsuitism ,irregular menses and signs of hyperprolactinemia. If results are normal, consider CAH, PCOS and anovulation. If prolactin levels are elevated then consider imaging of the pituitary gland and/or ovaries. Prolactin
Indicated in patients with hirsuitism ,irregular menses and signs of hyperprolactinemia. If results are normal, consider CAH, PCOS and anovulation. If prolactin levels are elevated then consider imaging of the pituitary gland and/or ovaries.
Serum androgen levels Measure total testosterone levels in patients with moderate or severe hirsutism having a normal menstrual history ,or hirsutism of any degree with sudden onset or rapid progression, or accompanied by signs/symptoms suggesting malignancy (abdominal/pelvic mass) or PCOS.
In the presence of a history of rapid virilization and a high testosterone level more than 1.5-2 times the upper normal limit, measure dehydroepiandrosterone sulphate (DHEA-S) level. A total testosterone level greater than 200 ng/dL would indicate an androgen-secreting ovarian tumor and a DHEA-S level greater than 700 μg/dL would indicate an adrenal cause (benign or malignant).
Serum androgen levels
Measure total testosterone levels in patients with moderate or severe hirsutism having a normal menstrual history ,or hirsutism of any degree with sudden onset or rapid progression, or accompanied by signs/symptoms suggesting malignancy (abdominal/pelvic mass) or PCOS.
In the presence of a history of rapid virilization and a high testosterone level more than 1.5-2 times the upper normal limit, measure dehydroepiandrosterone sulphate (DHEA-S) level. A total testosterone level greater than 200 ng/dL would indicate an androgen-secreting ovarian tumor and a DHEA-S level greater than 700 μg/dL would indicate an adrenal cause (benign or malignant).
Serum 17-hydroxyprogesterone level Indicated in patients with hirsutism having a normal menstrual history or in patients with irregular menses and inconclusive thyroid/prolactin level testing. 17-OHP is a unique serum marker for congenital adrenal hyperplasia. Testing should be done in the early follicular phase of the menstrual cycle in the morning (7-9 AM) . Levels less than 200 ng/dl rules out CAH and 12-hydroxylase deficiency. Levels greater than 200 ng/dl indicate a further ACTH stimulation test to be done. ACTH Levels less than 1000 ng/dl diagnoses the patient to be a heterozygote carrier of 12-hydroxylase deficiency and levels greater than 1000 ng/dl would diagnose the patient with CAH / 12-hydroxylase deficiency. Serum 17-hydroxyprogesterone level
Indicated in patients with hirsutism having a normal menstrual history or in patients with irregular menses and inconclusive thyroid/prolactin level testing. 17-OHP is a unique serum marker for congenital adrenal hyperplasia. Testing should be done in the early follicular phase of the menstrual cycle in the morning (7-9 AM) . Levels less than 200 ng/dl rules out CAH and 12-hydroxylase deficiency. Levels greater than 200 ng/dl indicate a further ACTH stimulation test to be done. ACTH Levels less than 1000 ng/dl diagnoses the patient to be a heterozygote carrier of 12-hydroxylase deficiency and levels greater than 1000 ng/dl would diagnose the patient with CAH / 12-hydroxylase deficiency.
24-hour urine cortisol /overnight low-dose dexamethasone suppression test /late-night salivary cortisol Should be measured in women with hirsuitism having signs and symptoms of Cushing syndrome. 24-hour urine cortisol /overnight low-dose dexamethasone suppression test /late-night salivary cortisol
Should be measured in women with hirsuitism having signs and symptoms of Cushing syndrome.
Somatomedin C (insulin-like growth factor 1) Test for acromegaly when suspected. Somatomedin C (insulin-like growth factor 1)
Test for acromegaly when suspected.
Ultrasound scan To be performed when PCOS, CAH or androgen-secreting tumours are suspected. Ultrasound scan
To be performed when PCOS, CAH or androgen-secreting tumours are suspected.
Pituitary MRI Must be performed if Cushing syndrome, hyperprolactinemia, or acromegaly is diagnosed by endocrinologic testing. Pituitary MRI
Must be performed if Cushing syndrome, hyperprolactinemia, or acromegaly is diagnosed by endocrinologic testing.

Investigations - Management

Fact Explanation
Repeat Ferriman-Gallwey scoring and take photographs of affected areas. For documentation of the response to treatment. Repeat Ferriman-Gallwey scoring and take photographs of affected areas.
For documentation of the response to treatment.
Retesting androgen levels To be retested after 3 to 6 months to document the response to treatment. Retesting androgen levels
To be retested after 3 to 6 months to document the response to treatment.

Management - Supportive

Fact Explanation
Patient education Hirsuitism causes a lot of anxiety and low self-esteem in women. Hence it is important to explain the underlying cause of the condition and provide support and information on hair removal methods and drug therapy. Patient education
Hirsuitism causes a lot of anxiety and low self-esteem in women. Hence it is important to explain the underlying cause of the condition and provide support and information on hair removal methods and drug therapy.
Home-based self-care management of excess hair Shaving, plucking, waxing, bleaching and use of depilatory creams. Home-based self-care management of excess hair
Shaving, plucking, waxing, bleaching and use of depilatory creams.
Clinical-based hair removal Electrolysis and laser therapy are available clinical methods which are more effective than home-based methods but more expensive. Clinical-based hair removal
Electrolysis and laser therapy are available clinical methods which are more effective than home-based methods but more expensive.
Lifestyle management and weight loss All obese women should be advised to lose weight. It an improve hirsuitism as well as reduce cardiovascular risk in obese patients with polycystic ovary syndrome (PCOS). Lifestyle management and weight loss
All obese women should be advised to lose weight. It an improve hirsuitism as well as reduce cardiovascular risk in obese patients with polycystic ovary syndrome (PCOS).

Management - Specific

Fact Explanation
Oral contraceptives(OCs)(One tablet daily ) First-line treatment for hirsuitism (mild,moderate,severe). Recommended pills are ethinyl estradiol (EE) with either 2mg of cyproterone acetate (Diane-35®, Schering) or 3mg drospirenone (Yasmin®, Bayer Healthcare). Oral contraceptives(OCs)(One tablet daily )
First-line treatment for hirsuitism (mild,moderate,severe). Recommended pills are ethinyl estradiol (EE) with either 2mg of cyproterone acetate (Diane-35®, Schering) or 3mg drospirenone (Yasmin®, Bayer Healthcare).
Anti androgens Recommended for patients with moderate to severe hirsutism and for those who are contraindicated for use of OCs.
*Spironolactone (SPA) (Aldactone®, Pfizer) (100-200 mg daily ) An aldosterone antagonist and androgen blocker. Contraindicated for patients with renal insufficiency, anuria, chronic renal impairment, hyperkalemia, pregnancy (risk of pseudohermaphroditism), and abnormal uterine bleeding. SPA is seen to cause menstrual alterations, hence it is best to combine it with oral contraceptive pills.
*Cyproterone Acetate (CA) - a progestin with antiandrogenic activity which is a effective treatment for hirsuitism and is available in combination with ethinyl estradiol (EE) (2 mg CPA and 35 μg EE/tablet).
*Flutamide (250-500mg/d ) - A nonsteroidal antiandrogen. Should not be used as first-line therapy for hirsuitism due to its propensity for severe hepatotoxicity.
Anti androgens
Recommended for patients with moderate to severe hirsutism and for those who are contraindicated for use of OCs.
*Spironolactone (SPA) (Aldactone®, Pfizer) (100-200 mg daily ) An aldosterone antagonist and androgen blocker. Contraindicated for patients with renal insufficiency, anuria, chronic renal impairment, hyperkalemia, pregnancy (risk of pseudohermaphroditism), and abnormal uterine bleeding. SPA is seen to cause menstrual alterations, hence it is best to combine it with oral contraceptive pills.
*Cyproterone Acetate (CA) - a progestin with antiandrogenic activity which is a effective treatment for hirsuitism and is available in combination with ethinyl estradiol (EE) (2 mg CPA and 35 μg EE/tablet).
*Flutamide (250-500mg/d ) - A nonsteroidal antiandrogen. Should not be used as first-line therapy for hirsuitism due to its propensity for severe hepatotoxicity.
Insulin-Sensitizing Drugs: Metformin (500-1000mg twice daily ) Useful for treating polycystic ovary syndrome. Provide little or no benefit for hirsutism symptoms, hence should not be used as a primary treatment for hirsutism. Insulin-Sensitizing Drugs: Metformin (500-1000mg twice daily )
Useful for treating polycystic ovary syndrome. Provide little or no benefit for hirsutism symptoms, hence should not be used as a primary treatment for hirsutism.
Glucocorticoids: Predisone (5-10mg daily ) Indicated for women who have hirsuitism that is due to non-classical congenital adrenal hyperplasia or have a poor response/ tolerance to OCs and/or antiandrogens; or for women who are seeking ovulation induction. Glucocorticoids: Predisone (5-10mg daily )
Indicated for women who have hirsuitism that is due to non-classical congenital adrenal hyperplasia or have a poor response/ tolerance to OCs and/or antiandrogens; or for women who are seeking ovulation induction.
5-RA inhibitor: Finasteride (Propecia) (2.5 mg daily ) - A potent inhibitor of the type 2 isoenzyme of 5-á-reductase, which blocks the conversion of testosterone to 5-á-dihydrotestosterone. It is found to be effective in the treatment of Idiopathic hirsuitism. 5-RA inhibitor: Finasteride (Propecia) (2.5 mg daily ) -
A potent inhibitor of the type 2 isoenzyme of 5-á-reductase, which blocks the conversion of testosterone to 5-á-dihydrotestosterone. It is found to be effective in the treatment of Idiopathic hirsuitism.
Gonadotropin-Releasing Hormone (GnRH) Agonists Indicated for patients with severe hirsuitism who don't respond to the OCs and antiandrogens. Suppresses LH and FSH (to a lesser degree) secretion leading to a decline in ovarian androgen production. Gonadotropin-Releasing Hormone (GnRH) Agonists
Indicated for patients with severe hirsuitism who don't respond to the OCs and antiandrogens. Suppresses LH and FSH (to a lesser degree) secretion leading to a decline in ovarian androgen production.
Eflornithine hydrochloride cream 13.9% (Vaniqa®, Skin Mediea) (Apply topically twice daily ) Topical Treatment - a biological modifier of hair follicular growth. Reduces unwanted facial hair in women. Recommended for mild hirsuitism. Eflornithine hydrochloride cream 13.9% (Vaniqa®, Skin Mediea) (Apply topically twice daily )
Topical Treatment - a biological modifier of hair follicular growth. Reduces unwanted facial hair in women. Recommended for mild hirsuitism.
Surgical management Required for androgen-secreting tumors causing hirsuitism in women. Surgical management
Required for androgen-secreting tumors causing hirsuitism in women.

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