Hirsutism (Androgen
Excess Syndrome)
Hirsutism is defined as
presence of excessive amount of coarse hair of male distribution seen in a
female, causing unacceptable cosmetic concern. Such areas include face, chest,
abdomen, thighs and back, etc. A semi-quantitative analysis of hirsutism can be
made according to Ferriman D and Gallway JD Nine body areas of hormone
sensitivity for hair growth are graded from 1 to 4 (minimal hair to frank virilization)
and ultimately the grades in each of these areas are summed together. If the
score is more that 8 it indicates hirsutism. The amount of hair growth differs
among various ethnic and genetic groups. It is much decreased in Orientals, for
example the Chinese and increased in Mediterranean’s. Hirsutism must not be
confused with virilization. Hirsutism does not always signify major pathology
or serious endocrine disturbance. In several hirsute women the disturbance is
due to excessive activity of 5 alpha
reductase in the skin. The enzyme releases dihydrotestosterone (DHT) from
testosterone carried along with sex hormone binding globulin (SHBG). Hirsutism
may also occur due to excessive sensitivity of hair follicles to DHT. This kind
of sensitivity is known as constitutional Hirsutism of no serious pathological
significance except that it is unacceptable to the patient. But hirsutism can
also be the earliest sign of a serious disorder like an androgen producing
tumor. Hirsutism of recent organ must be viewed with suspicion particularly if
the degree of hirsutism is increasing with time. This is due to androgen levels
increasing in the system caused probably by neoplasm. Frank virilization
characterized by clitoromegaly deepening of voice and alopecia may all follow
this kind of hirsutism.
Androgen excess
syndrome can occur due to several causes. Sixty to eighty percent of these
women are said to be having PCO syndrome. The syndrome is seen in 5 percent of
general population of reproductive age group. They may not all be infertile nor
will they all be hirsute. Forty to sixty percent of them are obese 50 to 90
percent are oligomenorrheic and 50 to 70 percent of them may have hirsutism of
low or high order. The high level of androgens and cyclical estrogen production
causes disruption of HT-HP gonadal axis resulting in elevated LH-FSH ratio.
Many women with PCO have metabolic disturbances some showing insulin resistance
and compensatory hyperinsulinemia with or without abnormal glucose tolerance (GT).
Some of the women with PCOS have dyslipidemia caused both by high levels of
insulin and androgens. Hirsutism caused by hyperinsulinemia may show at least a
partial response to administration of insulin sensitizing agents like
metformin.
HAIR –AN Syndrome: This
syndrome connotes a group of symptoms, which include hyperandrogenism, insulin
resistance and acanthosis nigricans. In this syndrome, the predominant feature
is hyperinsulinemia due to insulin resistance. The mitogenic action of insulin
on the basal cells of epididymis causes a velvety hyperpigmented patches or
streaks in skin creases. Although PCOS is also along with HAIR-AN syndrome the
degree of metabolic disturbance seen in HAIR-AN syndrome can be of a much
higher order.
Congenital Adrenal
Hyperplasia(CAH) is a disorder caused by deficiency of 21 hydroxylase enzyme
Cushing syndrome: The
clinical presentation of this syndrome is primarily that of abnormal fat
deposition of the body, thinning of skin with striae, glucose intolerance,
osteoporosis, muscle weakness along with all the other signs of PCO syndrome.
Androgenic tumors: Androgens
producing tumors of ovary or adrenal gland can cause hirsutism. But they are
rare. This should be suspected when the onset of symptoms are sudden and the
progression is rapid. Hormone producing tumors are usually of low malignancy.
When signs of excessive secretions of cortisol are present associated with virilization
one must look for adrenal tumors or secondary androgenism caused by PCO syndrome, etc. Unlike PCO or Cushingoid
pathology, virilising ovarian tumors
exhibit the form of sudden onset of hyper-androgenism with frank virilization.
These include Sertoli-Leydig cells and lipoid cell tumors.
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