Living with CAH
Coping with Hirsutism: What Treatment is Available for my Excess Hair?
This article is a collaborative project of professionals from diverse disciplines (under the direction of Drs. Maria I. New and Heino F. L. Meyer-Bahlburg) of New York Presbyterian, the combined University Hospitals of Cornell and Columbia.
Hair is really just an outgrowth of the skin layer called the epidermis. In fact, hair and skin are composed of the same protein (keratin). The hair shaft is produced by the hair follicle within the skin. The hair follicle has two regions: the hair bulb and the mid-follicle region. The hair bulb contains actively growing cells and pigment (melanin) producing cells. In the mid-follicle region the actively growing cells die and harden into what we call hair. The follicle can produce two types of hair: (1) vellus hairs, which are pale, fine, and silky; (2) terminal hairs, which are darker, coarser and larger.
During its life span, hair goes through three distinct phases: anagen, catagen and telogen. In the anagen phase, protein and keratin are continuously made to promote development and active growth of the hair shaft. At any point in time, 85-90% of hair is in the anagen phase, which can last from a few months up to six years. Hair then enters a transitional (or catagen) phase when chemical and structural changes cause the follicle to regress and stop growing. In the final part of the cycle, the telogen phase, the hair follicle shuts down and goes into resting mode. In this stage hair can shed so that new hair growth can begin. The telogen phase can last up to 100 days.
Hair growth in humans is asynchronous, meaning that growth and shedding of each follicle is independent of surrounding follicles. The number of hair follicles throughout the body is genetically determined. Men and women typically have the same number of hairs. However, in men, high androgen levels cause hair follicles in the androgen- sensitive areas of the body to produce terminal, coarser hair. In women, in whom androgen levels are typically low, those same hair follicles produce less visible, vellus hair.
When the delicate hormonal balance of the body is disturbed, hair production, among other things, can be affected. In the case of CAH, excess androgen (e.g., testosterone) production causes excess “terminal” hair to grow in the androgen-sensitive areas of the body. Excess hair can be categorized as either “hirsutism” or “hypertrichosis.”
Hirsutism indicates the presence of excess terminal hair growth in areas of the body that are androgen-sensitive. Androgen-sensitive areas of the body are the face, chest, areola, lower back, buttock, inner thigh and external genitalia. Hypertrichosis indicates excess terminal hair growth in areas of the body that are not androgen sensitive. Androgen-insensitive areas of the body include forehead, forearms, tops of the hands.
In women with hirsutism, the hair follicles that would normally produce pale, fine vellus hairs have switched to producing the darker, coarser terminal hairs. On the scalp the effect is the opposite, with hair follicles switching to production of vellus hairs in the presence of high androgen levels. This is why temporal balding is seen in untreated women with CAH. In general, hypertrichosis is not a problem in CAH since it does not depend on high androgen levels.
Endocrinologists use the Ferriman-Gallwey (FG) scale to assess the degree of hirsutism in CAH patients. Measuring the degree of hirsutism in 11 areas of the body, the FG scale can range from 0 – 44 (the higher the score, the more severe the hirsutism). A typical score for someone with hirsutism is between 8 and 29. While this scale is somewhat subjective, it does allow the physician to monitor the improvement in hair growth.
Treatment for Hirsutism
In patients with Congenital Adrenal Hyperplasia, excess androgen levels can cause an increase in terminal hair production. Therefore, the first step doctors take to treat hirsutism is to ensure that the patient is adequately controlled with replacement cortisol (cortef, prednisone or dexamethasone).
Even with optimal replacement of cortisol, however, there are certain times during the day when androgen levels rise, and, therefore, replacement cortisol alone is often not sufficient to combat hirsutism once it has begun. Elevated androgen levels can trigger a condition called “Polycystic ovarian syndrome” or PCO.
In PCO, the ovaries become enlarged with multiple cysts and begin to produce androgens. For this reason, if replacement therapy alone is not sufficient to combat hirsutism, you may be tested for PCO and, if found, oral contraceptives would be prescribed.
If hirsutism persists even with adequate replacement therapy and oral contraceptives, drugs which block androgen action may be tried. By blocking androgen action, terminal hair growth decreases with each passing cycle. However, significant improvement can take several months to two years because of the cyclical nature of hair growth. If the androgen blocker is discontinued, hair growth will recur.
Spironolactone is an androgen blocker which is also a weak diuretic (a drug that causes excess urination). While taking Spironolactone, electrolytes should be monitored periodically. Cyproterone acetate is another androgen blocker which is used in Europe and Australia but not approved in the United States. Cyproterone acetate also counteracts androgen action and is often combined with ethinyl estradiol (a type of estrogen) which counteracts the androgens produced by polycystic ovaries. The combination of cyproterone acetate and ethinylestradiol is found in Diane and Dianette. These drugs therefore operate as both an oral contraceptive and an androgen blocker at the same time.
Contraindications for cyproterone acetate (mainly due to the estrogen action) are varicose veins, uterine fibroids, smoking and cardiovascular disease. Side effects for both spironolactone and cyproterone acetate can include breast tenderness, decreased libido (sex drive), fatigue, headaches, depression, weight gain and irregular periods. In high doses, anti-androgens have been linked to liver toxicity. However, this is usually not a problem at the doses used to treat hirsutism. It is also extremely important that a woman does not become pregnant while on these medications as they will interfere with the normal development of a male fetus.
Flutamide, another anti-androgen medication, is no longer used by our center as other centers have reported two deaths tentatively associated with its use.
Hair Removal Methods
Bleaching: Many women use hydrogen peroxide to bleach their facial hair so that it is less noticeable. The only downside to this method is that sometimes the hair develops a yellow hue.
Shaving: This method offers temporary benefits and can sometimes cause skin irritation and infections. Many women have the mistaken belief that repeated shaving will cause the hair to grow in darker and coarser. After shaving, the only hairs that immediately regrow are the ones in the anagen phase.
Plucking: Plucking stimulates hairs that were in the telogen phase to begin their anagen phase. This means that plucking a hair that happens to be in the telogen phase actually speeds up the re-growth of that hair. Facial hair has a long telogen phase, so it is best to shave before plucking so that the only hairs plucked are already in the anagen phase.
Depilatory Creams: The most often used depilatory preparations contain thioglycolates, which target the keratin in hair. Since skin also contains keratin, depilatory creams often irritate the skin, sometimes causing dermatitis.
Vaniqa: A new cream called Vaniqa has been getting good reviews. Vaniqa is an enzyme inhibitor used topically to slow the growth of unwanted facial hair in women. Vaniqa must be prescribed by a physician.
Waxing: Waxing involves the application of warmed wax to hair-bearing skin. Upon cooling of the wax, hairs are imbedded within the wax and when the wax is pulled away in a quick motion, the trapped hairs are pulled away with it. Waxing can be painful and may cause hyperpigmentation, folliculitis, scarring and, if performed improperly, thermal burns.
Electrolysis: Electrolysis has been an option for over a century and is considered the only permanent form of hair removal. It uses an electrical current to disrupt individual hair follicles in the anagen (actively growing) phase. Since only a percentage of hair follicles are in the anagen phase at any given time, electrolysis must be done over several visits to steadily destroy all follicles in a given area. For instance, removing excess hair from the upper lip and chin could take approximately 18 monthly treatments, with the initial visits lasting longer than subsequent visits.
Most patients find it mildly uncomfortable, and some take anti-pain medication (e.g., Tylenol) before their appointment. For the minority of patients who experience electrolysis as painful there is the option of EMLA cream (for which you need a prescription from an MD) or LMX cream (over the counter but more expensive).
Some states require that electrologists be licensed. In these states we advise that you only use those who have a license. However, many states, including New York State, do not require electrologists to obtain a license. In these states it is recommended that that an electrologist be accredited by the American Electrology Association (AEA) and be a Certified Professional Electrologist (CPE). The AEA hosts a website at www.electrology.com with information about electrolysis and with a search engine to find AEA members in a given area.
Questions to Ask of an Electrologist when Choosing One
- Are you a CPE?
- Do you use disposable probes and do you sterilize your forceps?
- Is your equipment new (at least within the last 10 years)?
In unskilled hands, electrolysis can cause folliculitis (a painful, red swelling of the hair follicle) and scarring. Typical reactions include a mild redness that lasts for about one hour afterward, occasional breakouts, and minor temporary scabbing.
Many electrologists will require a doctor’s note if you have diabetes, are a pregnant woman, are on blood thinners, or have mitral valve prolapse (a common heart condition which usually causes no symptoms and doesn’t need to be treated).
Electrologists should offer a free consultation (where you can ask all these questions). They have variable fees, and, while electrolysis is not usually reimbursed by insurance, some people have successfully lobbied their insurance companies for reimbursement.
Laser Treatment: Laser treatment is the newest method of hair removal. There is less data on safety. Laser uses light waves to target the melanin (pigment) in the hair follicle and disrupt the follicle bulb. Because skin also contains melanin, light-skinned dark-haired individuals usually have the best outcomes with laser therapy. Like electrolysis, laser only destroys hairs in the anagen phase so multiple treatments are required to achieve hair removal in a given area. Approximately 50 – 70% of the excess hair can be removed. With this method follicles do not need to be treated one at a time, and therefore each treatment is relatively quick and usually only mildly uncomfortable.
There are several kinds of lasers used in treating hirsutism. The type of laser is chosen based on skin type. Laser treatment does not cause permanent hair loss since with time, hair tends to re-grow to a variable extent. This is why touch-ups are required on a yearly basis.
Complications could include permanent scarring and hypopigmentation (loss of normal skin color); however, based on very short term data, these risks appear to be low if the procedure is done by a trained dermatological surgeon.
At the moment there are no regulations governing the use of laser technology, and in fact it is offered in any number of settings from local spas to beauty parlors. We strongly recommend that laser treatment be done only by a dermatological surgeon. Surgeons should be a member of the American Society of Dermatological Surgery (ASDS) and have had specific medical training in the use of lasers. The ASDS hosts a website (www.asds.net) which contains information about laser hair removal and it provides a search engine which enables the user to find local ASDS approved dermatological surgeons.
Women with untreated CAH often experience temporal balding due to the action of androgens on the hair follicles of the scalp. Unlike the other androgen sensitive areas of the body, the hair follicles of the scalp respond to high androgen levels by making vellus (soft, pale and fine) hair instead of the usual terminal hair found on the scalp. Usually, when the underlying hormonal imbalance is treated with replacement cortisol (i.e., Cortef, prednisone or dexamethasone), women have a fairly rapid improvement in scalp hair re-growth.
In summary, there are many ways to battle hirsutism or hair loss. The first and most important step is to consult your endocrinologist and make sure that the replacement medication you are taking is adequate. Then, if hirsutism is the problem, discuss the various options presented here with your endocrinologist and/or dermatologist and find a treatment plan with which you are comfortable. If you choose electrolysis or laser therapy, your doctor may be able to recommend a well-trained local professional.