Advances in the evaluation and treatment of unwanted hair growth. (Cover Story).
Author/s: Ricardo Azziz
Whether the etiology is androgen excess or something more benign, excess hair growth negatively impacts quality of life for millions of women. A thorough evaluation is required to uncover underlying causes and provide appropriate treatment.
Estimates indicate that approximately 14 million women in the United States aged 30 to 59 years remove excess or unwanted facial hair at least once weekly. (1) In turn, hirsutism affects approximately 6% to 7% of reproductive-aged women. (2) Considering that there are about 52 million women aged 15 to 44 years in this country, we can estimate that there are approximately 3.5 million hirsute women of reproductive age in the US alone. (3)
Approximately 25% of women who complain of unwanted facial hair suffer from hirsutism, a majority of whom also have androgen excess. Hence, it is important to thoroughly evaluate every patient who complains of unwanted facial or body hair and not dismiss the problem as simply cosmetic. In addition, “cosmetic” problems can have severe and significant detrimental psychosocial effects on patients. Thus, problems with excess hair growth in women, whether true hirsutism or of a more benign nature, must be taken seriously since they significantly affect quality of life.
Defining the problem
Unwanted hair growth in women is a common and distressing problem that may signal an underlying endocrine or metabolic abnormality requiring diagnosis and treatment. The condition can present as either hirsutism or excess growth of vellus hair (that is, vellus hypertrichosis), and some women have unwanted body hair of the terminal type (coarse, thicker, and more pigmented) growing primarily in skin abnormalities such as congenital nevi.
Although vellus hypertrichosis is common in some ethnic groups, such as people of Mediterranean extraction, some women find the dark but relatively short and soft hairs cosmetically bothersome. While the majority of patients with this condition do not have an underlying hormonal disorder, vellus hypertrichosis occasionally can be the result of thyroid dysfunction, excess growth hormone, or ingestion of certain medications such as antiepileptic drugs.
Hirsutism is defined as the presence of terminal hairs on women in a male-like pattern and often is a sign of underlying androgen excess (hyperandrogenism). Other signs of androgen excess include acne, androgenic alopecia, and irregular ovulation and menstruation. Approximately 60% to 70% of hirsute patients suffer from the polycystic ovary syndrome (PCOS) (Table 1). In turn, patients with PCOS frequently have insulin resistance accompanying their hyperandrogenemia and are at increased risk for long-term metabolic complications including diabetes, hypertension, and potentially cardiovascular disease.
About 5% to 15% of hirsute patients suffer from idiopathic hirsutism (IH), which is characterized by the presence of hirsutism in the absence of either ovulatory dysfunction or elevated androgen levels. In contrast to the patient with PCOS, it is unlikely that a woman with IH will suffer from insulin resistance and long-term metabolic complications.
Depending on ethnicity and geographic location, from 2% to 8% of hirsute patients will suffer from 21-hydroxylase-deficient nonclassic adrenal hyperplasia (NCAH), and approximately 3% to 4% demonstrate severe insulin resistance consistent with the hyperandrogenic-insulin resistant-acanthosis nigricans (HAIRAN) syndrome. Ovarian and adrenal tumors as a cause of hirsutism in women are extremely rare, being present in 1:300 to 1:1,000 of such patients.
An increasingly more frequent cause of mild hirsutism is the ingestion of androgenic hormones. Over-the-counter products such as DHEA and androstenedione are androgenic and androgens may be prescribed for menopausal therapy, taken for treatment of endometriosis (danazol), and given in supplement form to induce growth of muscle mass in athletes.
Evaluating the patient with unwanted body or facial hair
Differential diagnosis includes history-taking, clinical exam, and laboratory testing, as necessary. Do not, however, let a family history of unwanted hair growth mislead you into considering the disorder “familial” and not requiring an evaluation. In fact, disorders such as PCOS, severe insulin resistance, and IH all have strong familial components and yet do require a thorough evaluation.
Taking the history. First, it is important to obtain a thorough history when evaluating the patient with unwanted hair growth. Take a menstrual and reproductive history, including a determination of when menses began (menarche), whether cycles are regular and predictable or irregular, or whether there any signs of premenstrual progesterone production (breast tenderness, swelling, or mood changes). In addition, you should determine whether the patient has had any prior problems conceiving and if ovulation induction was required. Because many women with PCOS suffer from premature adrenarche during childhood, you should ask the patient about the onset of pubic and axillary hair growth and breast development. Obviously, information regarding current drug intake, including use of “herbal or natural” supplements, should also be obtained.
It is also most important to understand the rate of progression of the hair growth and its severity. Seriously consider the possibility of an androgen-secreting tumor if the woman’s symptoms are relatively severe and their progression has been relatively rapid (that is, from nothing to her present condition in less than 2 years), or if they began very late in life. In fact, a thorough history and physical examination is the most sensitive tool for the prediction of such neoplasms. Also obtain a family history, and probe for instances of hair growth, irregular menstruation, diabetes, hypertension, or heart disease in first-degree relatives.
During the physical examination, it is important to determine whether the patient has isolated facial vellus hair overgrowth or whether the unwanted hair growth is part of a more extensive condition such as hirsutism. This determination will require an examination of the entire body as well as the face. If the hair growth pattern is suggestive of male-like distribution and type, the patient should be considered as having hirsutism. While hirsutism is often clearly observable on facial examination, it is not possible to determine the severity of the disorder simply by examining the face. (4) Some patients can have severe hirsutism of the face but minimal hirsutism on the remainder of their bodies. Conversely, minimal hirsutism of the face with extensive body hair growth suggestive of a more significant disorder also is possible. If examination reveals hair growth that is relatively fine, short, and minimally pigmented, the patient well may have vellus hypertrichosis. Nonetheless, remember that in the early sta ges of hirsutism (that is, in an adolescent patient) many of the hairs may still be in the preterminal stage (not yet “terminalized” or converted to terminal hairs), with an appearance similar to vellus hypertrichosis.
In addition to determining whether a patient has hirsutism, also examine her for related clinical features. Women with insulin resistance, for example, may have acanthosis nigricans and acrochordons. Acanthosis nigricans is a velvety darkly-pigmented change of the skin frequently seen around the nape of the neck and other crural areas of the body and acrochordons are small skin tags that present in these same areas. Other clinical features of importance to observe include the presence of alopecia, which in women can simply be represented by a diffuse thinning of the scalp hair in the sagittal and frontal areas, and the presence of acne, both facial and on the upper back, upper arms, and chest. These latter signs also suggest androgen excess.
You should also look for signs of Cushing’s syndrome and thyroid dysfunction. Assess the patient’s body fat distribution because significant abdominal obesity (the so-called “apple-shaped” body habitus) suggests insulin resistance and increased risk for diabetes and cardiovascular disease. Finally, the physical examination should include a pelvic exam, which will aid in determining whether the patient has clitoromegaly or any large or asymmetrical adnexal masses. While the presence of an androgen-secreting neoplasm is quite rare, clitoromegaly, unilateral adnexal mass, and general evidence of virilization are highly suggestive of this possibility.
Laboratory evaluation. Tailor your laboratory evaluation to the results of the patient’s clinical exam and the history-taking. If the clinical findings suggest simple vellus hypertrichosis and the patient is not taking any medications that may be associated with this problem, then a thyroid profile may suffice. Alternatively, in patients who have evidence of hirsutism, a more thorough evaluation is required to exclude related disorders. The evaluation should include a thyroid profile and an unstimulated 17-hydroxyprogesterone (17-HP) level to include thyroid disorder and 21-hydroxylase deficient NCAH, respectively. The serum level of 17-HP should be obtained in the morning and in the follicular phase (within 8 to 10 days of the beginning of menstruation or a progestin-induced withdrawal bleed).
If the level of 17-HP is greater than 2 ng/mL, then the patient should undergo an ACTH stimulation test to exclude the disorder. (5) If the 17-HP level during ACTH stimulation is greater than 10 to 12 ng/mL, then the diagnosis of 21-hydroxylase deficient NCAH is established. Alternatively, if the screening level is less than 2 ng/mL, 90% of patients with NCAH will be excluded. (6)
If the patient also has evidence of ovulatory dysfunction, a serum prolactin and fasting insulin and glucose levels also can be considered to exclude hyperprolactinemia and severe insulin resistance and diabetes, respectively While the prolactin level helps to exclude a related disorder that may be responsible for oligo-ovulation, the insulin and glucose measurements are used for excluding gross metabolic abnormalities in patients who may have PCOS.
It is important to note that one of the principal aims of the laboratory evaluation is to determine whether a patient actually has ovulatory dysfunction. Clearly, individuals who have menstrual abnormalities (oligomenorrhea) have ovulatory dysfunction, assuming the endometrial cavity is normal. Alternatively, up to 40% of hirsute women who have “regular menstrual cycles” also have oligo-ovulation Hence, any hirsute patient who claims to have “regular menstruation” should have her ovulatory function studied by having her keep a basal body temperature chart and/or obtaining a serum progesterone level on days 20 to 24 of the menstrual cycle. Progesterone levels greater than 4 ng/mL usually suggest ovulation.
The value of androgen levels in the evaluation of the hirsute patient is less clear. (8) Hirsutism per se is often a sufficient indicator of underlying “androgen excess,” particularly in women who have other reproductive abnormalities such as ovulatory dysfunction. While testosterone and DHEAS levels often have been suggested as good screening markers for the detection of ovarian and adrenal androgen-secreting neoplasms, respectively, clinical data clearly demonstrate that the history and physical exam is the single best predictor of such tumors. Furthermore, the vast majority of patients who have significantly elevated levels of both of these hormones, but do not have any overt clinical evidence of a tumor (that is, no virilization, rapid progression of the disorder, or Cushingoid features) generally do not have tumors. (9) Androgen levels actually may be most useful in the oligo-ovulatory patient who has mild hirsutism and/or acne because the measurements can confirm the presence of androgen excess.
Overall, 21-OH-deficient NCAH will be diagnosable by the screening or the ACTH-stimulated 17-HP level, while PCOS is a diagnosis of exclusion made in the hirsute patient with ovulatory dysfunction. IH, too, is a diagnosis of exclusion, defined as the presence of hirsutism in a patient who has no evidence of ovulatory dysfunction or elevated circulating androgen levels. (10) HAIRAN syndrome is diagnosed by the presence of hirsutism, ovulatory dysfunction, and hyper-androgenemia in the presence of severe and significant elevations of basal and/or glucose-stimulated insulin levels (basal insulin levels generally [greater than or equal to]80 [mu]U/mL and/or insulin levels following 75 g of oral glucose [greater than or equal to]300 [mu]U/mL). (11) Primary thyroid dysfunction and hyperprolactinemia are diagnosed by measuring circulating TSH and prolactin levels, respectively. The presence of virilization, rapid progression of the disease, or Cushingoid features suggests an androgen-secreting tumor, which can be di agnosed by either radiologic or surgical exploration.
Therapy for a patient with hirsutism focuses on androgen suppression, generally accompanied by peripheral androgen blockade. Obviously androgen suppression in patients with androgen-secreting neoplasms will be accomplished by surgical removal of the offending lesion. For other patients who have functional abnormalities of androgen excess, oral contraceptives (OCs) have significant value in suppressing ovarian androgen secretion. (12) Some patients with severe insulin resistance (HAIRAN syndrome) also may benefit from ovarian androgen suppression using long-acting GnRH-analogs. (13) More recently, insulin-sensitizing drugs such as metformin and the thiazolidinediones have been used to induce modest androgen suppression in patients with PCOS (Table 2).
Overall androgen suppression with OCs will stop further progression of hirsutism while only modestly affecting the hair currently present. OCs also will induce regular withdrawal bleeds, decreasing the risk of endometrial hyperplasia and cancer, and of dysfunctional uterine bleeding. It should be noted that glucocorticoid administration (dexamethasone) generally has minimal impact on hair growth and may result in a worsening of the insulin resistance and an increase in body weight. (14) Besides its use in treating patients with hyperandrogenism, androgen suppression also is beneficial for patients with IH.
In addition to receiving androgen suppression, the hirsute patient also should be treated with androgen blockade, unless her excess hair growth is minimal. (15,16) In the US, the principal medication used for this purpose is spironolactone, 100 mg to 200 mg daily in divided doses. Spironolactone is an aldosterone agonist used as a diuretic, and consequently its side effects include mild hypotension, polyuria, and fatigue. Furthermore, many patients will complain of dyspepsia and nausea. Nonetheless, overall, spironolactone is generally well tolerated.
Flutamide, currently approved for the treatment of prostate cancer, is another antiandrogen of benefit in the treatment of the hirsute patient. Doses range from 125 mg to 500 mg per day, in divided doses. Side effects generally are fewer than those of spironolactone, although this drug carries the rare potential of hepatic toxicity Both spironolactone and flutamide are androgen receptor blockers. Cyproterone acetate, available in Europe, is a progestin with significant androgen receptor-blocking activity Therapeutic doses are generally in the 10- to 60-mg/day range. Unfortunately this drug is not available in the US. Finally, finasteride, a 5[alpha]-reductase, can also be used for the treatment of hirsutism. Because all of these medications are potential teratogens, able to feminize a male fetus, they should be used in conjunction with adequate contraception, primarily OCs.
We should note that the patient with simple vellus hypertrichosis, unless it represents an early form of hirsutism, generally will not benefit from either OCs or androgen receptor blockers. Mechanical and/or cosmetic therapies for excess hair growth are the primary treatments in this setting.
Therapy for the patient with unwanted hair growth would not be complete without treatment to remove existing hair growth (Table 3). A number of methods have been reported to reduce unwanted terminal hair growth, including electrology and laser hair reduction. Electrology has been available for decades, and although prospective randomized trials are few, extensive clinical experience appears to indicate that it does afford permanent hair reduction in most patients. Laser hair reduction is a much newer modality, and although it does appear to offer less-permanent hair removal than electrology it provides significant hair reduction. Nonetheless, these methods do have their drawbacks. Electrology requires multiple and frequent treatment sessions that can be painful, depending on the location and extent of hair involvement. Likewise, laser hair removal has the potential for causing skin damage and is primarily effective in those patients who have dark hairs and lighter skin. Both of these methods can be quite cost ly in the long run. As such, the recent advent of newer topical therapies for reducing hair growth is of importance.
The role of eflornithine hydrochloride
Eflornithine hydrochloride, previously known as [alpha]-difluoromethylornithine or DFMO, has been approved by the Food and Drug Administration for unwanted facial hair growth (Figure 1). It is currently not approved for unwanted hair growth in other parts of the body, although there is no evidence to suggest that it would not be effective in these areas. Eflornithine HCl acts as an irreversible inhibitor of L-ornithine decarboxylase (ODC), an enzyme that catalyzes the synthesis of polyamines (putrescine, spermidine, and spermine from ornithine and cadaverine from lysine), which are small cationic molecules that may play a role in cell migration, proliferation, and differentiation.
ODC activity and expression appears to increase in anagen (growing) and decrease in telogen (shedding) hairs. (17) Furthermore, ODC is localized in the bulb, bulge, and shaft of the hair follicle and is associated with cell perforation in hair follicle development and growth. (18) Thus, ODC stimulates the production of polyamines from either ornithine or lysine, and the polyamines proceed to stimulate the proliferation of matrix cells in the bulge and bulb of the hair follicle, stimulating hair growth. Androgens increase ODC activity and expression in the human prostate and in rodent testes, assessory sex organs, and kidneys. Furthermore, ODC activity in rat epididymis and prostate cells is significantly reduced by either hypophysectomy (resulting in androgen suppression) or flutamide (androgen blockade). Thus, it would appear that tissue levels of ODC are upregulated by androgens, suggesting a mechanism by which androgens cause increased hair growth and differentiation to terminal hairs.
Systemic eflornithine has been used for the treatment of arseno-resistant Trypanosoma brucei gambiense, which causes African sleeping sickness. (19) However, a side effect of this treatment was observed to be significant hair loss. Furthermore, the systemic administration of eflornithine was found to markedly alter and decrease hair growth in sheep, an effect counteracted by the addition of spermidine in vitro. (20) Overall, eflornithine inhibits hair growth by targeting anagen (growing) hair follicles, irreversibly inhibiting ODC in the proliferative cells of the follicles. This results in a reduction in the production of polyamines, which leads to a reduction in the production of hair shaft proteins, slower hair growth, and a general miniaturization (the reverse of terminalization) of the hairs being produced.
Two randomized, double-blind, placebo-controlled studies including 594 women have demonstrated the effectiveness of topical application of 13.9% eflornithine HCl cream in reducing unwanted facial hair growth. (21) In these studies, patients were randomized to receive either eflornithine HCl cream 13.9% or placebo in a 2:1 ratio, respectively. The study medication was applied twice daily for 24 weeks, and an 8-week “no treatment” phase followed. The inclusion criteria included subjects who required removal of facial hair at least twice weekly and who had [greater than or equal to]5 terminal hairs per [cm.sup.2] on the chin and upper lip, as identified by video analysis. Exclusion criteria included patients with severe inflammatory acne, pregnancy, or nursing mothers. In these studies, approximately 60% of patients were white, 30% were African-American, and 7% were Hispanic. Patients ranged in age from 18 to 83 years.
The primary efficacy end point was the physicians’ global assessment (PGA), a four-point scale for evaluating improvement or worsening from baseline of the hair growth. Assessments were made by the treating physician 48 hours after shaving, at baseline and weeks 2, 4, 8, 16, 24, and 32 of treatment. Photographs could be used as an aid to assess changes from baseline. Secondary end points included a subject self-assessment, video analysis hair measurement, and safety parameters. The data demonstrated that by 8 weeks of therapy 58% of subjects had some overall improvement, while 32% of subjects had marked improvement or better (Figure 2). It should be noted the improvement in hair growth rapidly disappeared during the 8-week no-treatment observation period, indicating that continued use of this topical medication was required to maintain hair growth suppression.
Because these studies were primarily performed in dermatologic populations, little is known about the etiology of the unwanted facial hair growth of the patients enrolled. Nevertheless, it is safe to assume that a significant proportion of these women had hirsutism and/or PCOS. Overall, the study subjects’ self-assessments and the video analysis results confirmed the improvement in hair growth with this medication, observable by 8 weeks of therapy.
Adverse events with the use of eflornithine HCl, 13.9% during these studies included stinging of the skin, reported by 8% of patients compared to 2.5% of those on placebo; skin tingling in 3.6% of those on the drug compared to 1.5% for placebo; and a 2.8% incidence of rash compared to 0% in the placebo-treated women. There was no evidence that this medication either worsened or improved acne. Larger observational and open-labeled studies from Europe have continued to confirm these findings. (21-23)
Overall, eflornithine HCl cream, 13.9%, significantly improved hair growth in almost 60% of women with unwanted facial hair. Although FDA approval of this medication is for the removal of unwanted facial hair only, it is likely that this medication will also be effective in reducing hair growth in other parts of the body Nonetheless, it should be noted that the systemic absorption of this medication may increase if larger body areas are treated and that the relative safety of treating other body areas has not yet been established. (24) The clinical response is observable as early as 8 weeks of therapy and continued treatment is required to maintain improvement. If no benefit is observed after 4 months or more of therapy, treatment should be discontinued as it will not be effective. Eflomithine HCl does not remove hairs, but slows and miniaturizes the hairs that are present, such that they become much less visible and coarse. Adverse events are generally mild and occur in less than 10% of patients. Eflornithine HCl appears to be useful in patients with hirsutism and in those with simple vellus hypertrichosis.
More than 14 million women complain of unwanted facial hair growth, approximately one fourth of whom actually suffer from hirsutism. Evaluation of these patients should include a determination of whether they actually have hirsutism and have concomitant endocrine or metabolic abnormalities. The appropriate treatment for a woman who has unwanted hair growth may include hormonal therapy, but should also include mechanical and/or cosmetic means of removing hair growth. The newly approved eflomithine HCl cream, 13.9% is a useful adjuvant in reducing noticeable hair growth in affected women.
Dr. Azziz is a Professor in the Departments of Obstetrics and Gynecology and Medicine, The University of Alabama at Birmingham, Birmingham, Ala.
(1.) Data on file. Bristol-Myers-Squibb/NFO Research Group, Greenwich, Cone, 1999.
(2.) Knochenhauer ES, Key TJ, Kahsar-Miller M, Azziz R, et al. Prevalence of the polycystic ovary syndrome in unselected black and white women of the Southeastern United States: a prospective study. J Clin Endocrinol Metab. 1998:83:3078-3082.
(3.) U.S. Census Bureau, Table NP-T3-A, July 2000 estimates.
(4.) Knochenhauer ES, Black V, Hines G, Azziz R, et al. Examination of the chin or lower abdomen only for the prediction of hirsutism. Fertil Steril. 2000:74:980-983.
(5.) Azziz R, Hincapie LA, Knochenhauer ES, et al. Screening for 21 -hydroxylase deficient non-classic adrenal hyperplasia among hyperandrogenic women: a prospective study. Fertil Steril. 1999;72:915-925.
(6.) Azziz R, Zacur HA. 21-Hydroxylase deficiency in female hyperandrogenism: screening and diagnosis. J Clin Endocrinol Metab. 1989:69:577-584.
(7.) Azziz R, Waggoner WT, Ochoa T, et al. Idiopathic hirsutism: an uncommon cause of hirsutism in Alabama. Fertil Steril. 1998:70:274-278.
(8.) Azziz R. The time has come to simplify the evaluation of the hirsute patient. Fortil Steril. 2000;74:870-872.
(9.) Waggoner W, Boots LR, Azziz R. Total testosterone and DHEAS levels as predictors of androgen-secreting neoplasms: a populational study. Gynecol Endocrinol. 1999:13:394-400.
(10.) Azziz R, Carmina E, Sawaya ME. Idiopathic hirsutism. Endocr Rev. 2000:21:347-362.
(11.) Moller DE, Cohen O, Yamuguchi Y, Azziz R, et al. Prevalence of mutations in the insulin receptor gene in subjects with features of the type A syndrome of insulin resistance. Diabetes. 1994:43:247-255.
(12.) Azziz R, Gay F. The treatment of hyperandrogenism with oral contraceptives. Seminars in Reproductive Endocrinology. 1989;7:246-254.
(13.) Azziz R. The hyperandrogenic-insulin-resistant acanthosis nigricans syndrome: therapeutic response. Fortil Steril. 1994:61:570-572.
(14.) Azziz R. Glucocorticoid suppression in the treatment of androgen excess. In: Azziz R, Nestler JE, Dewailly D, eds. Androgon Excess Disorders in Women. Lippincoft-Raven Pub: Philadelphia, Pa: 1997:737-746.
(15.) Venturoli S, Marescalchi O, Colombo FM, et al. A prospective randomized trial comparing low dose flutamide, finasteride, ketoconazole, and cyproterone acetate-estrogen regimens in the treatment of hirsutism. J Clin Endocrinol Metab. 1999:84:1304-1310.
(16.) Moghetti P, Tosi F, Tosti A, et al. Comparison of spironolactone, flutamide, and finasteride efficacy in the treatment of hirsutism: a randomized, double blind, placebocontrolled trial. J Clin Endocrinol Metab. 2000:85:89-94.
(17.) Ogawa H, Huttori M. Regulation mechanisms of hair growth. Curr Probl Dermatol. 1983:11:159-170.
(18.) Nancarrow MJ, Nesci A, Hynd Pi, et al. Dynamic expression of ornithine decarboxylase in hair growth. Mech Dev. 1999:84:161-164.
(19.) Pepin J, Milord F, Guern C, et al. Difluoromethylornithine for arseno-resistant Trypanosoma brucel gambiense sleeping sickness. Lancet. 1987:2:1431-1433.
(20.) Hynd Pl, Nancarrow MJ. Inhibition of polyamine synthesis alters hair follicle function and fiber composition. J Invest Dermatol. 1996:106:249-253.
(21.) Schrode K, Huber F, Staszak J, et al. and the Eflornithine Study Group. Randomized, double-blind, vehicle-controlled safety and efficacy evaluation of eflornithine 15% cream in the treatment of women with excessive facial hair. 58th Annual Meeting American Academy of Dermatology, San Francisco, Calif., March 10-15, 2000. Abstract #P291.
(22.) Schrode K, Huber F, Staszak J, et al., and the Eflornithine Study Group. Evaluation of the long-term safety of eflomithine 15% cream in the treatment of women with excessive facial hair. 58th Annual Meeting American Academy of Dermatology, San Francisco, Calif., March 10-15, 2000. Abstract #P294.
(23.) Hickman JG, Huber F, Palmisano M. Human dermal safety studies with eflornithlne HCI 13.9% cream (Vaniqa), a novel treatment for excessive facial hair. Curr Med Res Opin. 2001:16:235-244.
(24.) Malhotra B, Noveck R. Behr D. et al. Percutaneous absorption and pharmacokinetics of eflomithine HCI 13.9% cream in women with unwanted facial hair. J Clin Pharmacol. 2001:41:972-978.
[Figure 2 omitted]
Differential diagnosis of the hirsute patient
Etiology Prevalence (%)
Idiopathic hirsutism 5-15
21-OH NCAH 1-8
HAIRAN syndrome 3-4
Ovarian androgen-secreting tumors 0.3-0.1
PCOS–polycystic ovary syndrome
21-OH NCAH–21-hydroxylase deficient nonclassic adrenal hyperplasia
HAIRAN syndrome–hyperandrogenic-insulin resistant-acanthosis nigricans
Medical treatment of hirsutism: suppression of androgen production
Cosmetic treatment of hirsutism
RELATED ARTICLE: Key points
* Don’t be misled into thinking hirsutism is “familial” simply because a patient relates a family history of unwanted hair growth.
* Remember that examination of the face alone isn’t sufficient to determine the degree of hirsutism. Some patients have extensive growth of body hair but minimal facial hair, or vice versa.
* Evaluate ovulatory function in any hirsute patient who claims to have “regular menstrual cycles” to rule out oligo-ovulation.
* Consider treating hirsutism with hormonal therapy and also mechanical and/or cosmetic hair removal. Eflornithine HCI cream 13.9% may be useful, but only for removal of unwanted facial hair.
Coding tips for evaluation of unwanted hair growth and hirsutism
Appropriately reporting the encounter with the patient with excessive hair growth requires consideration of possible ICD-9 codes as well as CPT-4 codes. Let’s look at the diagnostic possibilities first.
Although clinical distinctions might be made between hirsutism and hypertrichosis, ICD-9 describes both conditions with ICD-9 code 704.1. There are multiple options for identifying other underlying conditions and signs and symptoms. The ICD-9 category 256, in the endocrine section of ICD, describes conditions related to ovarian dysfunction that may be helpful in describing the patient’s condition. The table below shows some useful options:
ICD-9 Code descriptor
256.1 Other ovarian hyperfunction (includes
hypersecretion of ovarian androgens)
256.39 Other ovarian failure (includes
delayed menarche, ovarian
primary ovarian failure
256.4 Polycystic ovaries
256.8 Other ovarian dysfunction
(used if more specific
ICD code not available)
There are numerous other specific and nonspecific possibilities depending on the signs, symptoms, and concomitant conditions exhibited by the patient. ICD-9 code 259.9, also in the endocrine section, describes an unspecified endocrine disorder and includes abnormalities in hormones. The ICD-9 category 626, in the section for the genitourinary system, lists disorders of menstruation including 626.4, which is defined as irregular menstrual cycle. Codes in the 790 section of ICD-9 describe nonspecific abnormal laboratory, radiologic, and clinical findings and are useful when studies are not conclusive. Particular conditions should be reported using the most specific ICD-9 code that best describes the circumstances. It may be helpful to first search for coding possibilities using the index. However, the codes should be verified by reviewing the main text in the ICD-9 manual.
Selecting the appropriate CPT code to describe the encounter with the patient requires the selection of both the appropriate category of service and level of care provided. If another physician or health-care professional sent the patient for an opinion, recommendation, or advice, then a code from the consultation series is likely appropriate. Outpatient consultations are reported using codes 99241 to 99245 depending on the extent of the history, examination, and medical decision-making involved. Since the first encounter probably includes at least a detailed history and examination, code 99243 might be appropriate. If the extent of the history and exam are more comprehensive, codes 99244 or 99245 are possible depending on the level of medical decision-making. Remember, the level of all three key components (history, exam, medical decision-making) must be met or exceeded when reporting consultation codes.
If the patient returns for follow-up, or if an established patient presents with complaints of unwanted bair growth, then an established outpatient code from the 99211 to 99215 series is reported. The level of service is easier to achieve for established patient encounters since only two of the three key components must be met or exceeded. Alternatively, if the majority of the encounter was spent counseling the patient regarding laboratory results or treatment options, then it might be best to select the level based on the time spent with the patient rather than the content of the key components. For example, if you spent a total of 25 minutes with the patient, and more than 50% of the time was spent in counseling/coordination of care activities, then the appropriate code would be 99214. This would be the case regardless of the extent of the history and exam. It is important to be sure that the total time spent with the patient is documented, along with an account of the discussion. The table below shows the associated times for consultations, new patient, and established patient codes.
Emily Hill, PA-C
Ms. Hill is a consultant an coding and compliance and President of Hill & Associates, Wilmington, N.C.
Outpatient-new 99201 99202 99203 99204
Times 10 minutes 20 minutes 30 minutes 45 minutes
Outpatient-established 99211 99212 99213 99214
Times 5 minutes 10 minutes 15 minutes 25 minutes
Outpatient consults 99241 99242 99243 99244
Times 15 minutes 30 minutes 40 minutes 60 minutes
Times 60 minutes
Times 40 minutes
Outpatient consults 99245
Times 80 minutes
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