Two conditions should be distinguished from hirsutism. Hypertrichosis is the term reserved for androgen-independent terminal hair in nonsexual areas, such as the trunk and extremities. This may be the result of an autosomal-dominant congenital disorder, a metabolic disorder (such as anorexia nervosa, hyperthyroidism, porphyria cutanea tarda), or medications (e.g., acetazolamide, anabolic steroids, androgenic progestins, androgens, cyclosporine, diazoxide, dehydroepiandrosterone (DHEA), heavy metals, interferon, methyldopa, minoxidil, penicillamine, phenothiazines, phenytoin, streptomycin, reserpine, valproic acid). Virilization is a marked and global masculine transformation that includes coarsening of the voice, increase in muscle mass, clitoromegaly (normal clitoral dimensions ± standard deviation [SD] are 3.4 + 1 mm width by 5.1 + 1.4 mm length) and features of defeminization (loss of breast volume and body fat contributing to feminine body contour) (1). Although hirsutism accompanies virilization, the presence of virilization indicates a high likelihood of more serious conditions than are common with hirsutism alone and should prompt evaluation to exclude ovarian or adrenal neoplasm. Although rare, these diagnoses become likely when onset of androgen effects is rapid or sufficiently pronounced to produce the picture of virilization.
The history should focus on the age of onset and rate of progression of hirsutism or virilization.
A rapid rate of progression or virilization is associated with a more severe degree of hyperandrogenism and should raise suspicion of ovarian and adrenal neoplasms or Cushing syndrome. This is true whether rapid progression or virilization occurs before, during, or after puberty. Anovulation, manifesting as amenorrhea or oligomenorrhea, increases the probability that there is underlying hyperandrogenism. Hirsutism occurring with regular cycles is more commonly associated with normal androgen levels and thus is attributed to increased genetic sensitivity of the pilosebaceous unit and is termed idiopathic hirsutism. When virilization is present, anovulation virtually always occurs.
In determining the extent of hirsutism, a sensitive and tactful approach by the physician is mandatory and should include questions regarding the use and frequency of shaving and/or chemical or mechanical depilatories. Typically, clinical evaluation of the degree of hirsutism is subjective. Most physicians arbitrarily classify the degree of hirsutism as mild, moderate, or severe. Objective assessment is helpful, especially in establishing a baseline from which therapy can be evaluated. The Ferriman–Gallwey Scoring System for Hirsutism quantitates the extent of hair growth in the most androgen sensitive sites. It is a scoring scale of androgen-sensitive hair in nine body areas rated on a scale of 0 to 4 (2). A total score higher than 8 is defined as hirsutism (Fig. 31.1) (3). Although widely used, this scoring system has limitations, one of which is the fact that the scale does not include the sideburn, buttocks, and perineal areas. Substantial hirsutism may be confined to one or two areas without exceeding the cutoff value in total hirsutism score. The score does not reﬂect the extent to which hirsutism affects a woman’s well being (3,4).