Update on the management of hirsutism

Cleveland Clinic Journal of Medicine. 2010 June;77(6):388-398 | 10.3949/ccjm.77a.08079

June 1, 2010|Cleveland Clinic Journal of Medicine

Shannon Harrison, MBBS, MMed, FACD;Najwa Somani, MD;Wilma F. Bergfeld, MD

ABSTRACTHirsutism is a source of significant anxiety in women. While polycystic ovary syndrome or other endocrine conditions are responsible for excess androgen in many patients, other patients have normal menses and normal androgen levels (“idiopathic” hirsutism). The goal of the evaluation is to rule out any underlying pathology. The goals of therapy are to treat any underlying condition and to remove the excess hair. Current options for hair removal are discussed. Educating patients so they have reasonable treatment expectations is essential, as significant improvement may take weeks or months, and treatment may need to be repeated on an ongoing basis.

KEY POINTS

The finding of polycystic ovaries is not required for the diagnosis of polycystic ovary syndrome, nor does their presence prove the diagnosis. Gonadotropin-dependent functional ovarian hyperandrogenism is believed to cause this syndrome; however, mild adrenocorticotropic-dependent functional adrenal hyperandrogenism also is a feature in many cases.
Even women with mild hirsutism with subtle symptoms and signs of hyperandrogenism can have elevated androgen levels, and thus, they deserve a laboratory evaluation.
Laser treatment does not result in complete, permanent hair reduction, but it is more effective than shaving, waxing, and electrolysis, producing partial hair reduction for up to 6 months.

A THOROUGH HISTORY IS CRITICAL TO DIAGNOSIS

A thorough medical history can provide important diagnostic clues in women with hirsutism. The clinician should elicit details about the onset and progression of the hair growth,^12,15 previous treatments, and any cutaneous signs of hyperandrogenism, such as acne, seborrhea, acanthosis nigricans, or patterned hair loss.

Also important are the menstrual history and a history of infertility. Primary amenorrhea is defined as failure to menstruate by 16 years of age if secondary sexual characteristics have developed, or by 14 years of age if no secondary sexual characteristics have developed, and it can indicate nonclassical congenital adrenal hyperplasia.

The clinician should also try to determine if the patient has a history of galactorrhea or symptoms of virilization (eg, deepened voice, clitoromegaly, increased muscle mass); a family history of hirsutism, polycystic ovary syndrome, HAIR-AN syndrome, metabolic conditions such as type 2 diabetes mellitus, or cardiovascular disease^12,15; or a history of symptoms of any condition known to produce hirsutism, such as Cushing disease, acromegaly, or a thyroid disorder. Also important is a drug history to determine if the patient has taken drugs such as androgens, anabolic steroids, or valproic acid (Depakote).²⁰

THE PHYSICAL EXAMINATION

Figure 1. A modification of the Ferriman-Gallwey scoring system. A score of 8 or more indicates hirsutism.

The physical examination involves use of the Ferriman-Gallwey hirsutism scoring system or a modified Ferriman-Gallwey scoring system (Figure 1), which helps categorize the severity and distribution of excess hair growth. A Ferriman-Gallwey score of 8 to 15 (out of a possible 36) indicates moderate hirsutism, whereas a score above 15 indicates severe hirsutism.^2,15

Another proposed predictor of hirsutism is that terminal hair on the chin or the lower abdomen (Ferriman-Gallwey score ≥ 2) is nearly 100% sensitive and 27% specific at predicting total-body hirsutism.²⁴

As part of the physical examination, the clinician should also look for other cutaneous signs of hyperandrogenism, such as acne, androgenetic alopecia, and seborrhea. Acanthosis nigricans is a sign of insulin resistance. Height and weight should be measured and the body mass index calculated. Blood pressure should be recorded, as high blood pressure may be seen in Cushing syndrome and is an important cardiovascular risk factor. Signs of virilization should be identified. Indicators of Cushing disease such as striae, moon facies, fat redistribution, fragile skin, and proximal myopathy should be noted as well as signs of thyroid disease, such as textural skin changes, goiter, and hair loss. Expressible or spontaneous galactorrhea suggests hyperprolactinemia. Acromegaly is associated with coarse facies and enlarged hands and feet. Many of the endocrinopathies can be caused by a pituitary adenoma, which can manifest as a visual field defect, so visual fields should be examined.²⁵ The examination should also exclude any palpable ovarian or adrenal mass.¹²

WHEN IS ADDITIONAL TESTING NEEDED?

Laboratory and radiologic testing in patients with hirsutism may be needed to confirm the diagnosis of a suspected underlying condition or to exclude serious underlying pathology (Table 1).^{1,12,15–18,20,22,23,25–28}

The current Endocrine Society guidelines²⁰ recommend obtaining an early-morning testosterone blood level in the following patients:

Women with moderate or severe hirsutism
Women with hirsutism of any degree with sudden onset or rapid progression, or accompanied by signs or symptoms suggesting malignancy or polycystic ovary syndrome: eg, menstrual irregularity, infertility, central obesity, clitoromegaly, or acanthosis nigricans.^15,20

Testing androgen levels in mild, isolated hirsutism has not been proven to be useful or to alter management.²⁰

Free testosterone level

An early-morning total or free testosterone level is the initial test in the laboratory evaluation of hirsutism.^12,15 Additional specialized laboratory testing may be needed to determine the free testosterone level,¹⁵ as the free testosterone test is not available at all laboratories. A normal total testosterone level does not exclude hyperandrogenism but can suggest the diagnosis of idiopathic hirsutism.¹⁵

Further testing is needed if the total testosterone level is normal or only slightly elevated, or if there is a strong clinical suspicion of an underlying condition such as endocrinopathy or tumor. It is also useful in patients whose hirsutism responds poorly to medical treatments¹⁵ (see discussion below).

If the total testosterone level is elevated, if the hirsutism is moderate to severe, if there are associated symptoms, or if hirsutism is acute or progressive, a further endocrinologic workup is needed,¹⁵ possibly including measurement of free testosterone, sex hormone-binding globulin, DHEAS, and androstenedione.¹⁵ Free testosterone, unbound to sex hormone-binding globulin, is the biologically active fraction, with the levels of binding globulin increased by drugs such as oral contraceptives¹⁵ and decreased by high insulin levels in insulin resistance.²⁵

Test in patients with mild hirsutism?

Although the guidelines suggest that no additional workup is necessary for women with mild hirsutism, we evaluate all patients with hirsutism and those with the SAHA clinical spectrum by measuring free and total testosterone and DHEAS. In our experience, even women with mild hirsutism with subtle symptoms and signs of hyperandrogenism and mild hirsutism often have elevated androgen levels.

Test in women with idiopathic hirsutism?

In women with idiopathic hirsutism, minor forms of functional ovarian and adrenal hyperandrogenism are believed to play a role and are thought to be undetectable with conventional testing.²⁵ The gonadotropin-releasing hormone (GnRH) analogue stimulation test may uncover occult hyperandrogenism in this setting, but it is used as a research tool and does not currently have application in routine clinical practice.¹⁴

It is important to remember that some women with apparent idiopathic hirsutism and a history of regular menstrual cycles are actually oligo-ovulatory or anovulatory. In these instances, another diagnosis should be considered,¹³ and referral to an endocrinologist for further evaluation of ovulatory function is recommended.¹³

References

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