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). [3]
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. [3] PCOS occurs in women of reproductive age. [4] 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. [1]
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. [3]
Menstrual and reproductive history Determine any history of infertility. [1] Primary amenorrhea can indicate nonclassical congenital adrenal hyperplasia. Presence of irregular menses may indicate benign causes of hirsuitism. [3]
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. [1]
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. [2] Intake of anabolic steroids by female atheletes and body builders may experience hirsuitism and signs of virilzation. [5]
Flow of milk from breast Expressible or spontaneous galactorrhea indicates hyperprolactenemia. [1]
Mood and sleep disturbances, fragile skin and weight gain Indicates Cushing syndrome. [1]
References
  1. HARRISON S., SOMANI N., BERGFELD W. F.. Update on the management of hirsutism. Cleveland Clinic Journal of Medicine [online] December, 77(6):388-398 [viewed 23 May 2014] Available from: doi:10.3949/ccjm.77a.08079
  2. MARKOVSKI M, HALL J, JIN M, LAUBSCHER T, REGIER L. Approach to the management of idiopathic hirsutism Can Fam Physician [online] 2012 Feb, 58(2):173-177 [viewed 23 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279274
  3. SACHDEVA S. HIRSUTISM: EVALUATION AND TREATMENT Indian J Dermatol [online] 2010, 55(1):3-7 [viewed 23 May 2014] Available from: doi:10.4103/0019-5154.60342
  4. MELISSA H. HUNTER, and PETER J. CAREK. Evaluation and Treatment of Women with Hirsutism. Am Fam Physician [online] 2003;67:2565-72. [viewed 25 May 2014] Available from: http://www.aafp.org/afp/2003/0615/p2565.pdf
  5. KICMAN AT. Pharmacology of anabolic steroids Br J Pharmacol [online] 2008 Jun, 154(3):502-521 [viewed 26 May 2014] Available from: doi:10.1038/bjp.2008.165

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). [3]
Acne, acanthosis nigricans, patterned hair loss, and seborrhea These are cutaneous signs of hyperandrogenism and acanthosis nigricans is a sign of insulin resistance. [1]
Swelling of the clitoris, deepening of the voice, acne, excessive facial and body hair Signs of virilization. [1]
Striae, moon facies, fat redistribution, fragile skin, supraclavicular fat pad and proximal myopathy Indicates Cushing syndrome. [2]
Textural skin changes, goiter, and hair loss Indicates thyroid disease. [2]
Expressible or spontaneous galactorrhea Indicates hyperprolactinemia. [2]
Coarse facies and enlarged hands and feet Indicates Acromegaly. [2]
Visual field defect Suggests a pituitary adenoma. [2]
Palpable abdominal or pelvic mass Suggests an adrenal or ovarian mass. [1]
References
  1. BODE D, SEEHUSEN DA, BAIRD D. Hirsutism in women. Am Fam Physician [online] 2012 Feb 15, 85(4):373-80 [viewed 23 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/22335316
  2. HARRISON S., SOMANI N., BERGFELD W. F.. Update on the management of hirsutism. Cleveland Clinic Journal of Medicine [online] December, 77(6):388-398 [viewed 23 May 2014] Available from: doi:10.3949/ccjm.77a.08079
  3. SACHDEVA S. HIRSUTISM: EVALUATION AND TREATMENT Indian J Dermatol [online] 2010, 55(1):3-7 [viewed 23 May 2014] Available from: doi:10.4103/0019-5154.60342

Differential Diagnoses

Fact Explanation
Hypertrychosis Generalized increase in growth of the hair on all of the body and not just in a male pattern distribution. [2]
Drug-induced hirsuitism Hirsutism. Patient may have used exogenous pharmacologic agents such as OCs, danazol, testosterone, anabolic steriods, metoclopramide, methildopa, phenothaizines, reserprine prior to onset which are known to cause hirsuitism. [2]
Idiopathic hyperandrogenism Hirsutism. Regular menses. Normal androgen levels and no features suspicious for other causes of hirsuitism. [2]
Idiopathic hirsuitism Hirsutism. Clinically and biochemically positive for hyperandrogenism but with regular menses. [2]
Polycystic ovary syndrome (PCOS) Hirsutism. No signs of virilization. Menstrual dysfunction, acanthosis nigricans and acne. Clinical or biochemical evidence of hyperandrogenemia. Metabolic syndrome (obesity (body mass index > 30 kg/m2), insulin resistance, hyperlipidemia, type 2 diabetes, lipid abnormalities, cardiovascular disease). Polycystic ovaries seen on ultrasonography. (Testosterone - Normal/Increased. LH/FSH - Normal/Increased. Increased LH:FSH ratio.) [1] [3]
Nonclassic congenital adrenal hyperplasia Hirsutism onset before puberty. Most common cause is 21-hydroxylase deficiency. No signs of virilization. (Testosterone - Normal/Increased. DHEAS - Normal. Cortisol - Normal/Decreased. 17-OHP - Increased. LH/FSH - Normal.) [1] [3]
Cushing syndrome Hirsutism. Mood or sleep disturbances. Striae, moon facies, weight gain, fat redistribution, fragile skin, proximal myopathy, supraclavicular fat pad, hypertension and insulin resistance. Elevated cortisol levels. Cushing syndrome is often associated with hyperandrogenism, particularly in those cases caused by adrenal tumors. [1] [3]
Acromegaly Hirsutism. Coarse facies, enlarged hands and feet. Visual field defect. Elevated Somatomedin C level. [1] [3]
Androgen-secreting ovarian tumour Hirsutism (acute onset, severe, or progressive), virilization, or a palpable abdominal or pelvic mass. (Testosterone - Normal/Increased. DHEAS - Normal. Cortisol - Normal. 17-OHP - Normal. LH/FSH - Normal/Increased.) [1] [3]
Androgen-secreting adrenal tumour Hirsutism (acute onset, severe, or progressive), virilization, or a palpable abdominal or pelvic mass. (Testosterone - Increased. DHEAS - Increased. Cortisol - Increased. 17-OHP - Normal. LH/FSH - Normal.) [1] [3]
Hyperprolactenemia Hirsutism. Expressible or spontaneous galactorrhea. Visual field defects if pituitary in origin. Elevated prolactin level. Maybe due to hypothalamic disease or a pituitary disease. [1] [3]
Thyroid disease Hirsutism. Hot or cold intolerance, textural skin changes, goiter, and diffuse scalp hair loss. [1] [3] Abnormal thyroid function tests would confirm a thyroid disease as the cause for hirsuitism. [2]
HAIR-AN syndrome (hyperandrogenism, insulin resistance, acanthosis nigricans) Hirsutism. Irregular menstrual cycle, infertility, weight gain and acanthosis nigricans. Metabolic syndrome (obesity, insulin resistance, type 2 diabetes, lipid abnormalities, cardiovascular disease). [1] [3]
References
  1. SACHDEVA S. HIRSUTISM: EVALUATION AND TREATMENT Indian J Dermatol [online] 2010, 55(1):3-7 [viewed 23 May 2014] Available from: doi:10.4103/0019-5154.60342
  2. MARKOVSKI M, HALL J, JIN M, LAUBSCHER T, REGIER L. Approach to the management of idiopathic hirsutism Can Fam Physician [online] 2012 Feb, 58(2):173-177 [viewed 23 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279274
  3. HARRISON S., SOMANI N., BERGFELD W. F.. Update on the management of hirsutism. Cleveland Clinic Journal of Medicine [online] December, 77(6):388-398 [viewed 23 May 2014] Available from: doi:10.3949/ccjm.77a.08079

Investigations - for 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. [3]
Luteinizing hormone (LH) /follicle-stimulating hormone (FSH)/ estradiol To evaluate infertility and ovulatory dysfunction. [4] An increased LH:FSH ratio (> 3) is a common finding in PCOS. [1]
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. [3]
Thyroid function tests Indicated in patients with hirsuitism ,irregular menses [5] and signs of thyroid disease. Measure thyroid-stimulating hormone (TSH) levels, free thyroxine, and thyroid peroxidase antibodies. [4] Abnormal results indicate thyroid dysfunction. If results are normal, consider CAH, PCOS and anovulation. [5]
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. [5]
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). [4]
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. [1]
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. [1]
Somatomedin C (insulin-like growth factor 1) Test for acromegaly when suspected. [4]
Ultrasound scan To be performed when PCOS, CAH or androgen-secreting tumours are suspected. [3]
Pituitary MRI Must be performed if Cushing syndrome, hyperprolactinemia, or acromegaly is diagnosed by endocrinologic testing. [4]
References
  1. SACHDEVA S. HIRSUTISM: EVALUATION AND TREATMENT Indian J Dermatol [online] 2010, 55(1):3-7 [viewed 23 May 2014] Available from: doi:10.4103/0019-5154.60342
  2. BODE D, SEEHUSEN DA, BAIRD D. Hirsutism in women. Am Fam Physician [online] 2012 Feb 15, 85(4):373-80 [viewed 23 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/22335316
  3. MARKOVSKI M, HALL J, JIN M, LAUBSCHER T, REGIER L. Approach to the management of idiopathic hirsutism Can Fam Physician [online] 2012 Feb, 58(2):173-177 [viewed 23 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279274
  4. HARRISON S., SOMANI N., BERGFELD W. F.. Update on the management of hirsutism. Cleveland Clinic Journal of Medicine [online] December, 77(6):388-398 [viewed 23 May 2014] Available from: doi:10.3949/ccjm.77a.08079
  5. MELISSA H. HUNTER, and PETER J. CAREK. Evaluation and Treatment of Women with Hirsutism. Am Fam Physician [online] 2003;67:2565-72. [viewed 25 May 2014] Available from: http://www.aafp.org/afp/2003/0615/p2565.pdf

Investigations - Followup

Fact Explanation
Repeat Ferriman-Gallwey scoring and take photographs of affected areas. For documentation of the response to treatment. [1]
Retesting androgen levels To be retested after 3 to 6 months to document the response to treatment. [1]
References
  1. HARRISON S., SOMANI N., BERGFELD W. F.. Update on the management of hirsutism. Cleveland Clinic Journal of Medicine [online] December, 77(6):388-398 [viewed 23 May 2014] Available from: doi:10.3949/ccjm.77a.08079

Management - General Measures

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. [1]
Home-based self-care management of excess hair Shaving, plucking, waxing, bleaching and use of depilatory creams. [1]
Clinical-based hair removal Electrolysis and laser therapy are available clinical methods which are more effective than home-based methods but more expensive. [1]
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). [1]
References
  1. HARRISON S., SOMANI N., BERGFELD W. F.. Update on the management of hirsutism. Cleveland Clinic Journal of Medicine [online] December, 77(6):388-398 [viewed 23 May 2014] Available from: doi:10.3949/ccjm.77a.08079

Management - Specific Treatments

Fact Explanation
Oral contraceptives(OCs)(One tablet daily [4]) 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). [3]
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 [4]) 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. [2] *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). [2] *Flutamide (250-500mg/d [3]) - A nonsteroidal antiandrogen. Should not be used as first-line therapy for hirsuitism due to its propensity for severe hepatotoxicity. [3]
Insulin-Sensitizing Drugs: Metformin (500-1000mg twice daily [4]) 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. [4]
Glucocorticoids: Predisone (5-10mg daily [4]) 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. [3]
5-RA inhibitor: Finasteride (Propecia) (2.5 mg daily [4]) - 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. [2]
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. [2]
Eflornithine hydrochloride cream 13.9% (Vaniqa®, Skin Mediea) (Apply topically twice daily [4]) Topical Treatment - a biological modifier of hair follicular growth. Reduces unwanted facial hair in women. Recommended for mild hirsuitism. [2]
Surgical management Required for androgen-secreting tumors causing hirsuitism in women. [1]
References
  1. HARRISON S., SOMANI N., BERGFELD W. F.. Update on the management of hirsutism. Cleveland Clinic Journal of Medicine [online] December, 77(6):388-398 [viewed 23 May 2014] Available from: doi:10.3949/ccjm.77a.08079
  2. SACHDEVA S. HIRSUTISM: EVALUATION AND TREATMENT Indian J Dermatol [online] 2010, 55(1):3-7 [viewed 23 May 2014] Available from: doi:10.4103/0019-5154.60342
  3. ALSANTALI A, SHAPIRO J. Management of hirsutism. Skin Therapy Lett [online] 2009 Sep, 14(7):1-3 [viewed 23 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20039595
  4. BODE D, SEEHUSEN DA, BAIRD D. Hirsutism in women. Am Fam Physician [online] 2012 Feb 15, 85(4):373-80 [viewed 23 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/22335316