Hirsutism with an unknown cause

Information on hirsutism and hair removal treatments
Cosmetic hair removal
Laser hair removal
Electrolysis
Finasteride
Oral contraceptives
Spironolactone
Flutamide
Cyproterone acetate
Eflornithine
Ketoconazole
GRH analogs
Metformin


 
Idiopathic Hirsutism

Hirsutism is a hair growth problem in women characterized by unwanted and excess hair production. Hirsutism is caused by three main reasons:

  1. Nonandrogenic factors are the one’s that are not related to disproportionate androgen activity
  2. Result of androgen excess
  3. Idiopathic hirsutism

Since androgens are the main hormones that affect hair production and development in humans, the most common cause of hirsutism is traced to androgen malfunction. Idiopathic hirsutism is the second most common cause of hirsutism after Polycystic Ovarian Syndrome (PCOS), which is associated with androgen dysfunction. Idiopathic hirsutism accounts for approximately five to 17 percent of hirsute cases.

Idiopathic hirsutism can be defined as excess terminal hair production in a male-like pattern in androgen-receptive body parts of patients who show no signs of endocrine or androgen disorders. This kind of hirsutism occurs in the presence of regular ovulation and normal androgen levels.

In hirsute patients, one of the crucial tasks is to conduct a through examination in order to distinguish idiopathic hirsutism from other forms of hirsutism. Though a lot more research needs to be done about its pathophysiology, patients with this kind of hirsutism have a probable excess peripheral 5a-reductase action in skin and hair follicle, other variations in androgen metabolism or greater sensitivity of the androgen receptor.

To understand any kind of hirsutism it is first necessary to know a bit about human hair biology.

Human hair formation process

Human hair follicles first form in a human fetus. The number of human hair follicles that grow, which is 3-5 million (20% of which is in the scalp), is genetically predetermined. To understand hirsutism better, one needs to know the three human hair types and their life cycle.

  1. Lanugo hair: This soft, downy hair is first formed in the fetal stage in the mother’s womb and is lost in late gestation or early postpartum stage.
  2. Vellus hair: It is non-pigmented, soft and short and occurs in the seemingly hairless body regions.
  3. Terminal hair: This is pigmented, dense, coarse and longer than vellus hair and composes the eyebrows, eyelashes, scalp hair, the pubic hair and axillary hair, etc.

The permanent conversion and development of vellus to terminal hair is a normal physiological process triggered by androgens, testosterone and dihydrotestosterone (DHT) in body areas that are androgen sensitive. This cycle normally begins at puberty, continues through adult life and gradually lessens with age and reproductive capacity in both sexes.

The normal life cycle of hair is made up of three alternating stages:

  1. Anagen stage: Growth
  2. Catagen phase: Involution
  3. Telogen phase: Rest

Symptoms and evaluation of idiopathic hirsutism

Primary symptoms of idiopathic hirsutism are excess terminal hair growth in androgen-sensitive body areas. However, menses (i.e. ovulation) and circulating androgen levels remain unaffected.

Moreover, studies about this kind of hirsutism found altered functioning of androgen receptors and altered androgen metabolism. It was found that in the peripheral blood lymphocytes of certain patients with idiopathic hirsutism, the longer of the two androgen receptor alleles was “preferentially methylated” (and hence dysfunctional). Hence, experts suggest that it is probable that genetic modifications of the androgen receptor function and possibly 5a-reductase functions can alter the manifestation of hirsutism. Around 40 per cent of eumenorrheic hirsute women show signs of anovulation and hence they are diagnosed for polycystic ovary syndrome (PCOS) and not idiopathic hirsutism.

Clinical screening for idiopathic hirsutism

Idiopathic hirsute patients must be evaluated with the following medical tests:

  • A daily basal body temperature charting
  • Luteal phase progesterone, DHEAS, testosterone
  • Either follicular phase basal 17-OH progesterone levels or a Cortrosyn (ACTH) stimulation III test

The diagnosis of this kind of hirsutism is also often done by a clinical exclusion in hirsute cases where the serum androgen level and ovulatory process seems normal.

Treatment of Idiopathic hirsutism

The treatment of any kind of hirsutism must aim to stop or at least slow down the formation of new terminal hair growth without destroying the existing hair follicles. For a comprehensive treatment plan, the hirsute patient must first get rid of terminal hairs. The treatment span will be determined by the rate of check of hair growth according to the anagen, catagen and telogen phases of the life cycle of hair. Approximately, at least 6 months of therapy is necessary to see some positive results.

Generally, the treatment procedure must include these three steps:

  1. Androgen suppression if it involves androgen excess problem
  2. Peripheral androgen blockade
  3. Mechanical/cosmetic removal and destruction of the unwanted hairs

The various permutations and combinations that can result in this three-pronged treatment are as follows:

  • Oral contraceptive pills (OCPs) and spironolactone and antiandrogen: They target androgen activity at hair follicles.
  • Flutamide and finasteride treatment have similar results as spironolactone.
  • Metformin and pioglitazone are insulin-sensitizing agents that can also help in treating hirsutism and acne.
  • Cosmetic and mechanical hair removal like bleaching or chemical depilation, plucking, waxing, shaving and more permanent procedures like laser therapy, electrolysis etc.

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