Dermatologic Manifestations of Hirsutism Clinical Presentation

In women, hirsutism exceeding culturally normal levels can be as distressing an emotional problem as the loss of scalp hair. The onset of hirsutism can take one of several forms. For example, in women with familial hirsutism, it often appears during puberty. Hirsutism usually develops gradually in patients with PCOS and CAH. Hirsutism appears abruptly when an androgen-secreting tumor arises.

A woman with hirsutism has excess terminal hair in a masculine pattern, but note that hirsutism may be difficult to evaluate in women who have blond hair.

A quantitative method of measuring hair growth, the Ferriman-Gallwey model, allows for the determination of the severity of hirsutism by assessing the extent of hair growth in 9 key anatomic sites, as follows:

• Face (particularly, moustache, beard, and temple areas; see the image below)The patient has late-onset congenital adrenal hyperplasia. She has clinical features similar to those found in polycystic ovarian syndrome, including hirsutism, acne, obesity, diabetes, and menstrual irregularities.
• Chest
• Areolae
• Linea alba
• Upper back
• Lower back
• Buttocks
• Inner thighs
• External genitalia

Other accompanying signs and symptoms may include some of the following:

• Acanthosis nigricans
• Obesity
• Pelvic mass
• Signs or symptoms of virility
• Signs or symptoms of Cushing syndrome
• Acne
• Alopecia

Ovarian causes of hirsutism

PCOS is a disorder that affects androgen levels. The most common cause of androgen excess and hirsutism is PCOS. Virilization is minimal, and hirsutism is often prominent. Characteristic features include menstrual irregularities, dysmenorrhea, occasional glucose intolerance and hyperinsulinemia, and, often, obesity. The hyperinsulinemia is believed to hyperstimulate the ovaries into producing excess androgens. Women with PCOS may show other cutaneous manifestations of androgen excess in addition to hirsutism, such as recalcitrant acne, acanthosis nigricans, and alopecia on the crown area of the scalp (a pattern that contrasts with the bitemporal and vertex androgenic alopecia seen in men). See Polycystic Ovarian Syndrome for more information.

Hirsutism may also be seen in women with the following ovarian conditions, most of which are associated with virilization:

• Luteoma of pregnancy
• Arrhenoblastomas
• Leydig cell tumors
• Hilar cell tumors
• Thecal cell tumors

Familial hirsutism

Familial hirsutism is not associated with androgen excess. Familial hirsutism is both typical and natural in certain populations, such as in some women of Mediterranean or Middle Eastern ancestry. See the image below.

Drug-induced hirsutism

Drugs that can induce hirsutism by their inherent androgenic effects include dehydroepiandrosterone sulfate (DHEA-S), testosterone, danazol, and anabolic steroids. Currently used low-dose oral contraceptives are less likely to cause hirsutism than were previous formulations.

Drugs such as phenytoin, minoxidil, diazoxide, cyclosporine, streptomycin, psoralen, penicillamine, high-dose corticosteroids, metyrapone, phenothiazines, acetazolamide, and hexachlorobenzene presumably exert their effects independently of androgens. The exact mode of action of these drugs on hair follicles is not known, but the same mechanisms do not appear to be involved in all patients.

Drug-induced hirsutism can be distinguished from drug-induced hypertrichosis, in which a uniform growth of fine hair appears over extensive areas of the trunk, hands, and face and is unrelated to androgen-dependent hair growth.

Adrenal causes of hirsutism

CAH in children (ie, the classic form of adrenal hyperplasia) may cause hirsutism. These children may be born with ambiguous genitalia, symptoms of salt wasting, and failure to thrive. Additionally, they may develop masculine features. See Congenital Adrenal Hyperplasia for more information.

Late-onset CAH usually occurs as an incomplete version of CAH and affects approximately 1-5% of women who are hyperandrogenic. In patients with late-onset CAH, hirsutism (without salt-wasting symptoms) may not develop until adulthood.

Signs of virilization and menstrual irregularities may not be observed until puberty or adulthood. Patients have clinical features that resemble PCOS.

Hirsutism and oligomenorrhea suggest 21-hydroxylase deficiency (elevated 17-alpha-hydroxyprogesterone). Another uncommon disorder is 3-beta-, 11-hydroxysteroid dehydrogenase deficiency (elevated 3-beta-, 11-hydroxysteroid levels), which may result in early- or late-onset CAH. See 3-Beta-Hydroxysteroid Dehydrogenase Deficiency for more information.

Cushing syndrome is a noncongenital form of adrenal hyperplasia characterized by an excess of adrenal cortisol production. The excessive growth is predominantly vellus (non–androgen dependent) hair.

Other causes

Less common but potentially serious disorders that may be associated with hirsutism include anorexia nervosa, acromegaly, hypothyroidism, hyperprolactinemia, and porphyria.

Idiopathic hirsutism or end-organ hirsutism occurs in a small proportion of women with hirsutism. Neither a familial form nor any detectable hormonal abnormality usually is diagnosed. Such patients have normal menses, normal-sized ovaries, no evidence of adrenal or ovarian tumors or dysfunction, and no significant elevations of plasma testosterone or androstenedione. Antiandrogen therapy may improve hirsutism in some idiopathic cases, which suggests that this form may be androgen induced. One theory is that many of these women may have mild or early PCOS and androgen levels in the upper-normal ranges. Eventually, idiopathic hirsutism probably may be recognized as a more subtle form of hypersecretion of hormones from the ovary or, possibly, the adrenal gland. A 2009 study found significant insulin resistance in nonobese patients with idiopathic hirsutism.^[2]