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Hirsutism is defined as the presence of excessive growth of hair in locations where hair growth in women is normally minimal or absent. Although it may be the initial sign of a serious underlying disorder, hirsutism by itself is usually benign and is frequently associated with PCOS. In contrast, virilization is the combination of hirsutism plus other signs of masculinization, such as clitorimegaly, deepening of the voice, temporal balding, decreased breast size, and loss of female body hirsutism, virilization, hersutism, hirsute, excessive hair Virilization is less common than hirsutism and is more often associated with a potentially serious disorder such as an ovarian or adrenal tumor. Hirsutism is usually associated with normal or slightly elevated levels of serum androgens, whereas virilization is associated with markedly increased androgen production by the ovary or adrenal (or both) and hirsutism, virilization, hersutism, hirsute, excessive hair
The number of hairs per unit area of skin is determined by genetic factors and is the same for both sexes of a similar ethnic background. For example, women and men of Mediterranean descent tend to have more body hairs per unit area than do Asians. In both women and men, hair follicles cover the body except for the lips, palms of the hands, and soles of the feet. Hair follicles are of two types: vellus and terminal. In women, excess androgen production stimulates vellus hairs to develop into long, coarse, pigmented terminal hairs in most areas of the body except the scalp, where terminal hairs are converted to vellus hairs, eventually resulting in temporal balding. Androgens stimulate, whereas estrogens inhibit hair growth in
The primary mechanism leading to the development of hirsutism and virilization is increased secretion of androgens by the ovary or adrenal. Under normal circumstances the circulating testosterone level is derived from direct secretion by the ovary and by extraglandular conversion of androstenedione secreted by the ovary and the adrenal. Little, if any, testosterone is released by the adrenal, but the adrenal is the
In women with isolated hirsutism, 75% of circulating testosterone is from ovarian secretion. In the presence of increased levels of testosterone, TeBG levels are reduced, leading to increased free testosterone and an increased metabolic clearance rate for testosterone. In women with mild hirsutism who have ovulatory menstrual cycles and normal levels of testosterone, androstenedione, and
A frequent cause is ovarian dysfunction, and in this category the most common cause is PCOS. As discussed earlier, PCOS is diagnosed on clinical grounds, and the onset of hirsutism and other symptoms associated with the syndrome usually occurs at about the time of menarche. In most cases, the rate of progression of hirsutism is constant but slow. On rare occasion signs of virilization (e.g., clitorimegaly) may develop in women with PCOS; this usually occurs as a result of particularly high rates of testosterone secretion in women with stromal hyperthecosis, a condition in which islands of luteinized thecal cells are present in the ovarian stroma distant from ovarian follicles. Hyperthecosis is probably not a distinct entity but rather an exaggerated manifestation of PCOS. Women with
The association of hyperandrogenism, insulin resistance, and acanthosis nigricans (so-called HAIR-AN syndrome) constitutes a specific subset of PCOS or hyperthecosis. In women with type A insulin resistance resulting from intrinsic defects in the insulin receptor, the
Ovarian |
PCOS |
Hyperthecosis |
Neoplasms |
Sex cord tumors |
Germ cell tumors |
Hilar cell tumors |
Adrenal rest tumors |
Mixed germ cell and gonadal tumors |
Tumors with functioning stroma |
Pregnancy associated |
Luteoma |
Hyperreactio luteinalis |
Adrenal |
Congenital adrenal hyperplasia/adult-onset adrenal hyperplasia |
21-Hydroxylase deficiency |
11beta-Hydroxylase deficiency |
3beta-Hydroxysteroid dehydrogenase deficiency |
Neoplasms |
Adenomas |
Carcinomas |
Cushing's syndrome |
Drugs |
Phenytoin |
Diazoxide |
Anabolic steroids |
Progestagens (19-norsteroid derivatives) |
Danazol |
Idiopathic |
Miscellaneous |
Hyperprolactinemia |
Acromegaly Menopause |
Ovarian tumors may secrete a variety of hormones in addition to androgens, including estrogens, hCG, serotonin, and thyroxine. All choriocarcinomas, some dysgerminomas, and a few malignant ovarian teratomas secrete hCG. Primary ovarian carcinoids may produce
Adrenal virilization is most commonly caused by congenital adrenal hyperplasia (21-hydroxylase deficiency). In women the diagnosis is usually made at birth because of sexual ambiguity. Less severe forms are caused by mutations that impair the function of the gene less
Iatrogenic hirsutism may result from drug therapy. One cause of hirsutism, amenorrhea, and signs of virilization is the use of androgens for the purpose of body-building or in treatment of diminished libido or menopause. Other drugs that cause hirsutism include danazol, metyrapone, phenothiazines, phenytoin, diazoxide, and minoxidil. Menopausal
Age in Years | Incidence of | ||||||
---|---|---|---|---|---|---|---|
Tumor | Hormones Produced | Peak | Range | Malignancy (% ) |
Bilaterality (% ) |
Size in cm | Miscellaneous |
Sex cord-stromal tumors | |||||||
Granulosa-theca cell tumors | Estrogen, androgens, progestagens | 30-70 | <1-92 | 10-20 | 10-15 | <1->30 | Most common functioning ovarian neoplasms |
Androblastomas (Sertoli-Leydig cell tumors) | Androgens, estrogens | 20-40 | 4-84 | 20 | Rare | <5->25 | Most common virilizing tumors |
Lipid cell tumors | |||||||
Hilar cell type | Androgens | 45-75 | 4-86 | Rare | Rare | 0.5-15 | -- |
Adrenal cell type | Estrogens | 20-25 | 6-78 | 20 | Rare | 0.5-30 | Often associated with diabetes |
Germ cell tumors | |||||||
Dysgerminomas | Chorionic gonadotropin | 10-30 | 4-76 | 100 | 5-10 | 3-50 | -- |
Teratomas | |||||||
Carcinoids | Serotonin | 50-70 | 36-79 | Rare | Rare | <1-15 | Carcinoid syndrome may occur |
Stuma ovarii | Thyroxine | 30-60 | 21-69 | Rare | Rare | <5-20 | |
Mixed carcinoid and struma | Serotonin, thyroxine | 40-60 | 21-77 | Rare | None | <1-26 | May be clinically hyperthyroid (rare) |
-- | |||||||
Choriocarcinomas | Chorionic gonadotropin | 6-15 | 6-42 | 100 | Rare | >5 | -- |
Gonadoblastomas | Androgens, chorionic gonadotropin | 10-30 | 6-36 | 50 | 40 | <1->30 | Usually occur in male pseudohermaphrodites |
The diagnosis and evaluation of hirsutism require a careful history and physical examination as well as laboratory testing. Familial occurrence, age at onset, severity, and rate of progression are important. Hirsutism that begins at the expected time of puberty may be
The treatment of hirsutism is not ideal, in that cures are rarely possible. The therapeutic approach is directed first at decreasing the rate of androgen secretion or inhibiting androgen action in the pilosebaceous unit itself. Oral contraceptives are commonly used to treat ovarian causes of hirsutism. Other drugs that may be useful include LHRH analogues, antiandrogens (e.g., spironolactone), and
After androgen secretion or androgen action is reduced, additional physical methods to remove hair may be instituted. These include
Surgical removal of the ovaries may be indicated in women with hyperthecosis in whom childbearing is no longer desired and in whom
Because abnormal hair growth can be caused either by excess androgen secretion or by increased sensitivity to androgens, treatment is
The most commonly used therapy for hirsutism is the oral contraceptive pill. Oral contraceptives lower androgen levels primarily by
The most widely used antiandrogens for the treatment of hirsutism are spironolactone (100 to 200 mg/d by mouth) and cyproterone acetate (50 to 100 mg/d by mouth). Both act primarily to block androgen effects at the pilosebaceous unit by competing with testosterone for the androgen receptor. Cyproterone acetate also inhibits LH levels, whereas spironolactone inhibits CYP enzymes
Two glucocorticoids, dexamethasone (0.25 to 0.5 mg) and prednisone (2.5 mg), have been used for treatment of hirsutism of adrenal
LHRH analogues are potentially useful for treatment of hirsutism. Nasal, subcutaneous, and depot forms are available (see later discussion). Ketoconazole, an imidazole derivative, inhibits production of CYP-linked steroidogenic enzymes (principally
Inhibition of gonadotropin release to the point of hypogonadism may be produced by LHRH agonists or antagonists. The agonist gonadorelin requires continuous infusion, whereas long-acting agonists such as leuprolide acetate or antagonists can be given once daily.