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DESCRIPTION
Hirsutism is excessive growth of hair on the face and body. Idiopathic hirsutism is defined as the occurrence of excessive male pattern hair growth in women who have a normal ovulatory menstrual cycle and normal levels of serum androgens. It may be a disorder of peripheral androgen metabolism. It usually occurs gradually over an extended period of time.
The extent of normal hair growth varies between individuals, families and races, being more extensive in the Mediterranean and some Asian subcontinent populations. These variations in body hair in the normal population varies from no visible hair to extensive cover with thick dark hair. It is very difficult to determine the dividing line between normal and abnormal degrees of facial and body hair in the female. Soft vellous hair is normally present all over the body and this type of hair on the face and elsewhere is considered normal and is not sex-hormone dependant. Any excess in these regions is usually considered a mark of increased ovarian or adrenal androgen production.
It has been traditional to divide women with hirsutism into those with no elevation of serum androgen levels and no other clinical features (usually labeled 'idiopathic hirsutism') and those with an identifiable endocrine imbalance (most commonly polycystic ovary syndrome (PCOS) or rarely other causes). However in recent years it has become apparent that most women with 'idiopathic hirsutism' have some radiological or biochemical evidence of PCOS on more detailed investigation.
Familial or idiopathic hirsutism does occur, but usually involves a distribution of hair growth which is not typically androgenic. Similarly, non-androgen-dependant hair growth occurs with drugs such as phenytoin, diazoxide, minoxidil and cyclosporin. Iatrogenic hirsutism also occurs after treatment with androgens, or more weakly androgenic drugs such as progestagens or danazol.
Rarer, and more serious, endocrine causes of hirsutism and virilization include congenital adrenal hyperplasia, Cushing's syndrome and virilization tumors of the ovary and adrenal. All these should be considered in any patients with hirsutism.
A wide variety of ovarian and adrenal steroid hormone products and precursors are androgenic and hypersecretion of androgens from one or both of these endocrine organs is usually found in women with hirsutism. In addition, estrogens are converted to androgens in adipose (fat) tissue, which represents a further source of androgen excess in obese women. The response of the hair follicle to circulating androgens also seems to vary between individuals with otherwise identical clinical and biochemical features, and the reason for this variation in end-organ response remains poorly understood. Whatever the underlying pathology, hair has a long growing cycle with spontaneous variations and clinical changes are slow, both as hair develops and as it responds to therapy.
The complaint of hirsutism is common and often accompanied by severe anxiety and social stress. The following are important issues to consider.
- The extent and severity of hirsutism. This should be recorded objectively, ideally using a scoring system, to document the problem and to monitor treatment. The method and frequency of physical removal (such as shaving, plucking) should also be recorded. Most women who complain of hirsutism will have an objective excess of hair on examination, but occasionally a normal pattern of hair will be found (and appropriate counseling is then recommended).
- Age and speed of onset. Hirsutism related to PCOS usually begins around the time of the menarche and increases slowly and steadily in the teens and twenties. Rapid progression and prepubertal or late onset suggests a more serious cause.
- Accompanying virilization. Hirsutism due to PCOS may be severe and effect all androgen-dependant areas on the face and body. However, more severe virilization may include clitoromegaly (abnormal enlargement of the clitoris), frontal balding and other male phenotyping (male physical characteristics) implies substantial androgen excess, usually indicates a rarer cause other than PCOS.
- Menstruation. Most women with hirsutism will have some disturbance of their menstrual cycles. The greater the disruption the more likely it is that there is a serious cause.
- Weight. Many women with hirsutism are also overweight or obese. This worsens the underlying androgen excess and insulin resistance and inhibits the woman's response to treatment. It is an indication for appropriate advice on diet and exercise. In severe cases the insulin resistance may have a visible manifestation as acanthosis nigricans (brown-black, poorly defined, velvety hyperpigmentation on the skin) on the neck and in the axilla (armpit-underarms).
FREQUENT SIGNS & SYMPTOMS
Hair thickens and darkens and grows in a male pattern. A woman with the milder form of hirsutism may notice significant growth of hair that is mature (the same color as scalp hair) on the upper lip, side burns, chin, around the nipples, chest or lower abdomen). A woman with more advanced hirsutism will cause mature hair to grow on the upper back, shoulders, sternum, and upper abdomen. It most often begins during puberty. If hirsutism starts before or after puberty, the cause could be hormonal.
Irregular or no menstruation.
Darkened, thickened, velvet-like skin in armpits, groin or neck area.
Acne. Skin disruptions.
Easy bruising, weight gain in midsection or muscle weakness.
Leakage of milk or other fluid from nipples when the women is not breast-feeding.
Deepening of the voice (sometimes).
High blood pressure, high blood sugar or cholesterol problem.
Hirsutism prior to puberty or hirsutism that occurs suddenly.
Infertility problems (sometimes).
Increased muscle mass.
Hirsutism runs in families. If other female relatives have these symptoms, discuss it with your family practitioner.
CAUSES
Usually due to excessive production of androgens (male hormones) from the ovary or adrenal gland caused by some conditions, such as polycystic ovary syndrome or congenital adrenal hyperplasia.
Adrenal or ovarian tumor. Occasionally, the overproduction of androgen hormones is caused by an abnormality in the ovaries, the adrenal glands or the pituitary gland of the brain.
Hair follicles that are overly sensitive to male hormones. Health care providers do not know why this occurs.
Some medications can cause hirsutism. These medications include birth control pills, hormones and anabolic steroids (body building steroids). Other medicines may indirectly cause the body to manufacture extra androgen hormones. These include some medicines to treat nausea, schizophrenia, agitation, epileptic seizures, migraine headaches, bipolar disorder, aggression and high blood pressure, as well as estrogens and opiate medications.
Some excessive hair growth does not fit the pattern of growth triggered by androgen hormones (for example, hair between the eyes, on the forehead, on the temples or high on the cheeks of the face). This hair growth, called hypertrichosis, can be caused by thyroid problems or anorexia nervosa. It also can result from long-term use of certain medications, including the following steroid hormones: cyclosporin (Neoral, Sandimmune, SangCya), phenytoin (Dilantin), minoxidil (Rogaine), penicillamine (Cuprimine, Depen), as well as some dermatology medications that commonly are combined with ultraviolet light treatments.
Hirsutism seems to run in families. Genetics and racial backgrounds may determine normal to abnormal patterns of hair growth. Normal patterns of hair growth and hair distribution vary widely, determined mostly by racial background. For example, whites as a group have more facial and non-scalp hair growth than do blacks or Asians, and white women of Mediterranean heritage normally have more hair growth than do women from Nordic countries.
Idiopathic (no apparent cause).
RISK INCREASES WITH
Family history of hirsutism.
Dark-haired individuals, especially those of Hispanic, African-American, Mediterranean, or Indian ancestry.
Use of male hormones (androgens) or corticosteroid medications, birth control pills, hormones and some anti-hypertensive drugs.
Menopause or anovulation (failure to ovulate).
PREVENTIVE MEASURES
No specific preventive measures. However, if you are obese, you should try to obtain a normal weight for your height and body size through proper nutrition and exercise. Consult a nutritionist to assist you with a dietary plan. Avoid medications that are known to cause hirsutism.
EXPECTED OUTCOME
Diagnosis and treatment of any underlying cause can frequently halt further hair growth. Response to treatment is a slow process and may take 6 to 12 months. Most cases of hirsutism create a tendency to have lifelong excessive hair growth. However, most women respond to medical treatment if they continue the treatment several months or longer. A few cases of hirsutism (such as tumors that produce androgen hormones or tumors in the pituitary gland) can be cured with surgical treatment, radiation or both.
Excess hair may be eliminated by various cosmetic methods. But most times it is a temporary method of removing unwanted hair. These may limit hair regrowth in treated areas.
POSSIBLE COMPLICATIONS
Poor self image; may feel unattractive and find social interaction with other people difficult.
Infertility.
Hirsutism may be unresponsive to initial treatment.
TREATMENT
GENERAL MEASURES
DIAGNOSIS
A physical examination, laboratory studies, and possibly some imaging studies (CT scan or MRI) will aid in diagnosing any underlying cause of the hirsutism. Your health care provider will ask you about your medical history with special attention to your menstrual cycles. He or she also will examine you. If you have a normal cyclic pattern of menstrual periods, the hirsutism is most likely genetic (inherited). If your menstrual cycles are irregular and have always been irregular, the cause could be polycystic ovary syndrome. If the hirsutism and menstrual irregularity are news, you will need to be evaluated for a potentially more serious condition, such as a tumor of the ovary, adrenal glands or pituitary gland. This is especially important is you are skipping periods. If you have mild hirsutism and don't have any symptoms that suggest you are significantly overproducing androgen hormones, you may not need any additional testing.
Some possible tests that may be performed and aid in the diagnosis of women with hirsutism:
- Serum testosterone and dehydroepiandrosterone may be checked. Serum testosterone may be elevated in PCOS and is invariably substantially raised in virilization tumors. Women with hirsutism and normal testosterone level frequently have low levels of sex hormone binding globulin (SHBG), leading to high free androgen levels. SHBG can be measured. Other androgens such as androstenedione and DHEA sulphate are frequently elevated in PCOS, and even more elevated in congenital adrenal hyperplasia and virilizing tumors.
- 17-x-Hydroxyprogesterone is elevated in classical CAH (congential adrenal hyperplasia), but may be apparent in late-onset CAH only after stimulation.
- Gonadotrophin levels. LH hypersecretion is a consistent feature of PCOS, but the wide fluctuation in the secretion of this hormone means that an increased LH/FSH ratio is not always observed on a random sample.
- Estrogen levels. Estradiol is usually normal in PCOS, but estrone levels (which are rarely measured) are elevated due to peripheral conversion. Levels are variable in other causes.
- Ovarian ultrasound. The most consistent investigation in PCOS is ovarian ultrasound by a trained health care provider. The typical ultrasonic features are those of a thickened capsule, multiple 3-5mm cysts and hyperechogenic stroma. It should also be noted that prolonged hyperandrogenization from any cause may lead to polycystic changes in the ovary. Ultrasound may also reveal virilization ovarian tumors, although these are often small.
- Magnetic resonance imaging (MRI) of the brain or a computed tomography (CT) scan of the adrenal glands may be done.
- Serum prolactin. Mild hyperprolactinaemia is common in PCOS but rarely exceeds 1500mUL-1. Prolactin may be measured to check for signs of a tumor in the pituitary gland.
- Blood sugar and cholesterol levels may be tested, because diabetes and high levels of cholesterol commonly are associated with some cases of hirsutism.
- If a virilization tumor is suspected clinically or after investigation, then more complex tests may include dexamethosone suppression tests, CT or MRI or adrenals, and selective venouse sampling catheters.
Most women presenting with a combination of hirsutism and menstrual disturbances will be shown to have polycystic ovary syndrome, but the rarer alternative diagnoses should always be considered, and excluded with appropriate testing if suspected. This includes late-onset CAH (early-onset, raised serum 17-x-OH-progesterone), Cushing's syndrome (look for other clinical features) and virilization tumors of the ovary or adrenals. (severe virilization, markedly elevated serum testosterone).
The extent of investigative testing will depend on the particular woman and will be determined by her health care provider. In many cases a single serum testosterone may be sufficient to exclude rare causes. Urine free cortisol should be measured if Cushing's syndrome is a clinical possibility and 17-x-OH-progesterone if early onset or family history suggests congenital adrenal hyperplasia.
TREATMENT
The underlying cause should be removed in the rare instances where this is possible (such as drugs, adrenal or ovarian tumors). Other therapy depends upon whether the aim is to reduce hirsutism, regularize periods or produce fertility. The specific type of treatment will depend on the cause of the hirsutism. A mild case of hirsutism with no menstrual irregularities may require no treatment. For others, treatment sometimes depends on the patient's desire for future childbearing.
Ovarian or adrenal tumors should be surgically removed.
Cosmetic treatment choices of the excess hair include shaving, plucking, bleaching, waxing, or use of depilatories; however, shaving and plucking can cause infection or scarring. Waxing is helpful where the "bikini area" is causing the concern. Electrolysis will remove hair permanently, but it is a slow, expensive treatment.
Systemic therapy by the use of medicational treatment may be recommended. This always requires a year or more of treatment for maximum benefit, and long-term treatment is frequently required as the problem tends to recur when treatment is stopped. The woman must therefore be an active participant in the decision to use systemic therapy and must understand the risks as well as benefits.
- Estrogens, often given in the form of oral contraceptives, suppress ovarian androgen production and reduce free androgens by increasing SHBG levels when these are low. Combined pills, which contain a non-androgenic progestogen have a theoretical advantage over older combined pills, and will result in a slow improvement in hirsutism in a majority of cases and should normally be used first unless there is a contraindication.
- Cyproterone acetate (50-200 mg daily) is an anti-androgen but is also teratogenic and a weak glucocorticoid and progestogen. Given continuously it produces amenorrhea, and so is normally given for days 1-14 of each cycle. In women of child bearing age, contraception is essential.
- Spironalactone (200 mg daily) also has anti-androgen activity and can cause useful improvements in hirsutism in selected cases.
- Other agents of doubtful efficacy include bromocriptine and cimetidine.
- New agents which remain to be fully evaluated include finasteride and flutamide.
- Eflornithine Cream is the first topical prescription treatment for women with unwanted facial hair. Eflornithine works by inhibiting the growth of facial hair and was shown in controlled clinical trials to provide clinically meaningful and statistically significant improvement in the reduction of facial hair growth in women.
- Additional information available from:
Daughters of Hirsutism Association
203 N. LaSalle St. Suite # 2100
Chicago, IL 60601
(312) 558-1365
American Society for Reproductive Medicine
1209 Montgomery Highway
Birmingham, AL 35216-2809
(205) 978-5000
American Academy of Dermatology
P.O. Box 4014
Schaumburg, IL 60168-4014
Phone: (847) 330-0230
Toll-Free: (888) 462-3376
Fax: (847) 330-0050
www.aad.org
American College of Obstetricians and Gynecologists
P.O. Box 96920
Washington, DC 20090-6920
Phone: (202) 638-5577
www.acog.org
MEDICATION
There are no medicines specifically approved for treating hirsutism.
the most frequently used medication to treat hirsutism is a diuretic (water pill), but it seems to be the most effective at reducing hair growth. Other medicines that may be recommended for hirsutism caused by excess androgen production include dexamethsone, oral contraceptives, leuprolide and anti-androgens. They vary in effectiveness and take 3 to 6 months for results. They may help decrease new hair growth, but will usually not change the amount of hair you already have. There is also a medicine made specifically to slow down the growth of facial hair. It is a prescription cream that you apply to the affected skin on your face and chin. This medicine may start to work as soon as 4 to 8 weeks after you begin treatment.
Additional medications may be prescribed for any underlying disorder (see treatment above).
Shaving is the safest and easiest method of removing hair. However, you will have stubble unless you shave every day. Your skin may become irritated with frequent shaving. If skin becomes irritated from shaving, use non-prescription 1% hydrocortisone cream.
Depilatories or creams to remove hair are often recommended. They leave no stubble. Use with caution as they may irritate the skin. To test how sensitive your skin is, apply a small amount of cream to the inside of your wrist. Wait for one day before applying the cream to other parts of your body. If you do not have a bad reaction to the cream on your wrist, it is probably okay to use it.
Bleaching paste may also be used. Use this product according to the directions on the label. Bleaching products may irritate your skin.
Plucking and waxing can cause skin irritation and make the hair grow faster by increasing the blood supply to the follicle. These methods are not recommended for women who have hirsutism.
Electrolysis gets rid of hair permanently by delivering a small electrical current through a needle placed into the hair follicle. Electrolysis is expensive and time-consuming. If you choose to have electrolysis, make sure the operator is qualified and licensed. Home electrolysis products and electronic tweezers don't work well and are not recommended.
Laser hair removal uses a laser light to damage hair follicles so unwanted hair falls out. This also prevents the hair from growing back. You'll probably need multiple laser treatments over a number of weeks, and the results may not be permanent. Laser hair removal is very expensive and can only be done by a licensed practitioner. Side effects of the procedure may include redness, darkening or lightening of the skin, and scarring.
ACTIVITY
Usually no restrictions.
DIET
No special diet.
If overweight, a weight loss diet is usually recommended. Losing weight reduces the amount of hormones in your body that cause increased hair growth.
NOTIFY YOUR HEALTH CARE PROVIDER IF...
You or a family member has symptoms of hirsutism such as a sudden increase in facial or body hair, if your periods have become irregular or if your voice has become deeper.
New, unexplained symptoms develop. Drugs used in treatment may produce side effects.
You become pregnant. Some medicines used to treat hirsutism will need to be discontinued.
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