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Chapter 24 HIRSUTISM

Theodore X. O’Connell

General Discussion

The following discussion of hirsutism focuses on adolescent girls. For a discussion of hirsutism in infants or children, please see Chapter 35, Precocious Puberty.

Hirsutism is defined as the presence of excessive coarse terminal hair in a pattern not normal in females in areas such as the face, chest, or upper abdomen. This disorder is a sign of increased androgen action on hair follicles, which may result from increased levels of endogenous or exogenous androgens, or it may result from increased sensitivity of hair follicles to normal levels of circulating androgens. Hirsutism must be distinguished from hypertrichosis, which is the term used to describe the androgen-independent growth of hair that is vellus and does not follow the bodily distribution of the hair associated with male secondary sexual characteristics. Hypertrichosis may be familial or may be caused by medications or metabolic disorders.

Any patient who has either physical evidence of hirsutism or significant concern over excess hair growth should be evaluated. When evaluating hirsutism, it is important to determine whether hirsutism exists alone or whether virilization is also present. This distinction is important because virilization may reflect a serious underlying pathologic condition such as malignancy. Virilization manifests with a wide range of signs of androgen excess, such as acne, hirsutism, frontotemporal balding, amenorrhea, oligomenorrhea, deepening of the voice, and clitoromegaly.

The most common triggering factor for hirsutism is excess androgen production. Although androgens may come from an exogenous source, androgen excess is most commonly endogenous. The two primary sources of endogenous androgens are the adrenal glands and the ovaries. The most common cause of hyperandrogenism presenting in a teenage girl is polycystic ovary syndrome (PCOS). The differential diagnosis also includes late-onset congential adrenal hyperplasia, virilizing tumors, Cushing syndrome, hyperprolactinemia, acromegaly, medications, and abnormalities of androgen action or metabolism.

A common endocrine disorder, PCOS affects 3% to 10% of premenopausal women. It represents a group of disorders associated with increased androgen production from the ovaries, adrenal glands, or both. The clinical presentation typically includes one or several of the following features: hirsutism, obesity, oligomenorrhea, anovulation, and infertility. At the 1990 National Institutes of Health (NIH) consensus conference, experts attempted to identify the key features needed to diagnose PCOS, noting the following as definite criteria: hyperandrogenism, menstrual dysfunction, clinical evidence of hyperandrogenism, and the exclusion of congenital adrenal hyperplasia. Probable criteria included insulin resistance, perimenarchal onset, an elevated ratio of luteinizing hormone (LH) to follicle stimulating hormone (FSH), and polycystic ovaries by sonography.

Patients with PCOS must be differentiated from individuals with variants of nonclassic congenital adrenal hyperplasia (CAH). The most common form of CAH is late-onset 21-hydroxylase deficiency. The diagnosis of nonclassic CAH can be made definitively by the use of adrenocorticotropin hormone (ACTH)-stimulation testing or can be inferred by measurement of early morning 17-hydroxyprogesterone levels.