Hirsutism

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: ; Ogheneochuko Ajari, MB.BS, MS [2],Rasam Hajiannasab M.D.[3]

Hirsutism (from Latin hirsutus = shaggy, hairy) is defined as excessive and increased hair growth in women in locations where the occurrence of terminal hair normally is minimal or absent. It refers to a male pattern of body hair (androgenic hair) and it is therefore primarily of cosmetic and psychological concern. Hirsutism is a symptom rather than a disease and may be a sign of a more serious medical indication, especially if it develops well after puberty.It affects 5-15% of women . In most of the cases there is an underlying hormonal imbalance . Excessive amount of androgen plays a major role, as 70% of patients with excessive androgen will develop hirsutism. However in 5 to 15% of patients suffering from hirsutism , there is no increase in androgen level and are considered as idiopathic hirsutism. About 50% of hirsutism cases have high androgen level. Free testosterone is the main circulating androgen and is often elevated in hirsute women and the level of androgens and hair follicle sensitivity to androgens play the major role .^[1] Ovulatory dysfunction (PCOs), diabetes, thyroid hormone abnormalities and CAH are some of the underlying causes for hirsutism.^[2]There is a scoring system which is called  Ferriman–Gallwey scale, which quantitates the extent of hair growth in the most androgen-sensitive sites and patients with a score of 8 or more ,would be considered a hirstue .Hirsutism must be distinguished from hypertrichosis which is a result of either heredity or the use of medications such as glucocorticoids, phenytoin, minoxidil, or cyclosporine. Hypertrichosis is not caused by excess androgen.^[2]Pubertal onset hirsutism specially when it is mild ,points toward PCOS or idiopathic hirsutism but sever late onset hirsutim with other virlization signs can be due to ovarian or adrenal tumors. Hirsutism can lead to significant psychological distress for women and even depression if left untreated and based on the underlying cause other medical complications can occur (e.g. in cases of hirsutism due to PCOS , if the treatment doesn’t address PCOS , it can lead to infertility.) Prognosis depend on underlying etiology .Treatment options include: Cosmetic and hormonal therapy .Cosmetic therapy includes shaving, waxing , laser hair removal therapy , etc. Hormonal therapy includes : oral contraceptives, finestride , spironolactone ,etc.

Throughout the history hirsutism has been regarded as a syndrome of hair growth in women in a male pattern , obesity and menstural irregularity (Apert, 1910) . Other authors considered hirsutism as masculine hair growth only(Howard and Whitehill, 1937; Glass and Bergman, 1938). Hirsutism has been the most outstanding symptom in virilism and masculinization and also the major feature of adreno-genital syndrome which was introduced in 1905 by Bulloch and Sequiera.^[3]

Hirsutism is classified using 11 body areas to assess hair growth using the Ferriman–Gallwey score, a method of evaluating and quantifying hirsutism in women.

Androgens are essential for sexual hair and sebaceous gland development. Pilosebaceous unit (PSU) growth and differentiation require the interaction of androgen with numerous other biological factors. In the embryo the pattern of PSU responsiveness to androgen is determined. Hair follicle growth involves close reciprocal epithelial-stromal interactions that recapitulate ontogeny; these interactions are necessary for optimal hair growth in culture.^[4] Androgens are responsible for hair follicle size, hair fiber diameter, and the proportion of time terminal hairs spend in the anagen phase.^[5] Almost all hirsute women have an increased production rate of androgens specially testosterone. ^[6]In some women, an increased conversion of testosterone to dihydrotestosterone (DHT) by the enzyme 5-alpha-reductase, is responsible for hirsutism.^[7]

The cause of hirsutism can be either an increased level of androgens (male hormones) or an oversensitivity of hair follicles to androgens. Male hormones such as testosterone stimulate hair growth, increase size and intensify the pigmentation of hair. Other symptoms associated with a high level of male hormones include acne and deepening of the voice and increased muscle mass. Growing evidence implicates high circulating levels of insulin in women to the development of hirsutism. This theory is consistent with the observation that obese (and thus presumably insulin resistant hyperinsulinemic) women are at high risk of becoming hirsute. Further, treatments that lower insulin levels will lead to a reduction in hirsutism.

The diagnosis of hirsutism requires the exclusion of related disorders with peripheral androgen activity. Hirsutism starts shortly after puberty with a slow course and progression.

Hirsutism only affects women, since the rising of androgens causes a male pattern of body hair, particularly in locations where women normally do not develop terminal hair within their puberty (chest, abdomen, back and face). The medical term for excessive hair growth that affect both men and women is hypertrichosis.

Several factors increase the risk of developing hirsutism and an accurate history would aid in accurately identifying the risk.

Screening in all hirsute women should be done in order to detect possible malignant and concerning underlying causes .

Pubertal onset hirsutism specially when it is mild ,points toward PCOS or idiopathic hirsutism but sever late onset hirsutim with other virlization signs can be due to ovarian or adrenal tumors. Hirsutism can lead to significant psychological distress for women and even depression if left untreated and based on the underlying cause other medical complications can occur (e.g. in cases of hirsutism due to PCOS , if the treatment doesn’t address PCOS , it can lead to infertility.) Prognosis depend on underlying etiology .

There are no specific diagnostic criteria for hirsutism. Most women seek medical help for the inconvenience of the presence of hirsutism and clinical assessment is to determine the underlying cause.^[8]

The hallmark of hirsutism is excessive facial hair growth. A positive history of virilizing symptoms is suggestive of an underlying hormonal imbalance.

Patients with hirsutism usually appear normal and in no acute distress. The degree of hirsutism can be estimated using the Ferriman-Gallwey score.

Laboratory tests that should be done in hirsutism include testosterone level, DHEAS, and 24-hour cortisol level.

CT scan is helpful in the diagnosis of underlying adrenal or ovarian tumors.

Magnetic resonance imaging can be helpful in finding the underlying etiology of hirsutism.

Pharmacologic medical therapies for hirsituism include oral contraceptives, androgen receptor blockers, 5-alpha reductase inhibitors, gonadotrophin-releasing hormone (GnRH agonist), adrenal suppressive glucocorticoids, insulin-sensitising agents, and biological modifiers of hair follicular growth. Treatment options are systemic therapy and topical therapy.

Non-pharmacologic medical therapies for hirsituism include lifestyle modification and some cosmetic measures. Lifestyle modifications are majorly for polycystic ovary syndrome (PCOS) patients, include decreasing weight, exercise, diet, and smoking cessation. Cosmetic measures are classified as permanent methods, such as electrolysis or laser therapy, and transient home methods, such as pluking, shaving, waxing, chemical depilators, and bleaching.

The mainstay of treatment for hirsutism is medical therapy. Surgery is usually reserved for patients with either ovarian tumor, adrenal tumor, perimenopausal or postmenopausal women with severe hyperandrogenism who are candidated for oophorectomy, or bariatric surgery for severe obesity.

Primary prevention in hirsutism is focused on controlling the conditions that can lead to hyperandrogenism in women.

Effective measures for the secondary prevention of hirsutism include lifestyle modification and pharmacological prevention. The goal of secondary prevention is to prevent cardiovascular and metabolic diseases (diabetes mellitus).

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: , Rasam Hajiannasab M.D.[2], Ahmed Elsaiey, MBBCH [3]

In the course of the history, hirsutism has been described as a syndrome of male pattern hair growth, obesity and menstrual irregularity in women. Some authors described hirsutism as masculine hair growth only. However, in 1905 Bulloch and Sequiera described hirsutism in detail and explained the association of hirsutism, virilization, and masculinization along with the principal features of adreno–genital syndrome.

• In 1905, Bulloch and Sequeira were the first to report cases of pre-pubertal virilizing changes due to adrenal cortex lesions.^[1]
• In 1910, DR. Apert used the term of “Adrenal virilism” for hirsutism.
• In 1912, Dr. Gallais named hirsutism as “Adreno–genital syndrome”.^[1]
• Between 1933 and 1953, Dr. Broster and Vines held various studies on the adreno–genital syndrome. Dr. Broster described the adreno–genital syndrome as a disorder including hirsutism before or after puberty and Cushing’s syndrome.^[1][2]

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: ; Ogheneochuko Ajari, MB.BS, MS [2] Rasam Hajiannasab M.D.[3]

There is no established system for the classification of hirsutism. However, Ferriman Gallwey scoring system is used to assess the severity of hirsutism based on the degree of excess male pattern body hair. In this method, different body areas are determined to assess hair growth.

The Ferriman–Gallwey score is a method of evaluating and quantifying hirsutism in women. The method was originally published in 1961 by D. Ferriman and J.D. Gallwey in the Journal of Clinical Endocrinology.^[1] The original method used 11 body areas to assess hair growth, but was decreased to 9 body areas in the modified method:^[1]

• Upper lip
• Chin
• Chest
• Upper back
• Lower back
• Upper abdomen
• Lower abdomen
• Upper arms
• Forearms (deleted in the modified method)
• Thighs
• Legs (deleted in the modified method)

In the modified method, hair growth is graded/staged from 0 (no growth of terminal hair) to 4 (extensive hair growth) in each of the nine locations.

• Scores may vary from a minimum score of 0 to a maximum score of 36.
• In Caucasian women, a score of 8 or higher is considered to be due to androgen excess.
• The pattern and extension of hair growth on the body varies according to the ethnicity of the patient.

There were further modifications in 2001 to include a total of 19 locations.

• The added additional areas included in these modifications include:^[2]

• Sideburns
• Neck
• Buttocks
• Inguinal area
• Perianal area
• Forearm
• Leg
• Foot
• Toes
• Fingers
• Each area has its own specified definition of the four-point scale

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: ; Ogheneochuko Ajari, MB.BS, MS [2] Rasam Hajiannasab M.D.[3]

The growth of hair on sex-specific areas of the body occurs due to androgens. Androgens induce vellus follicles in sex-specific areas to develop into terminal hairs, which are larger and more heavily pigmented.

The growth of hair on sex-specific areas of the body occurs due to androgens. Androgens stimulate the growth of vellus follicles in sex-specific areas to develop into terminal hairs, which are larger and have increased pigmentation (darker).^[1][2][3][4]

• There are approximately 5 million hair follicles at birth in humans with about 80,000 to 150,000 of them on the scalp.
• The quality of hair is determined by hormones and other intrinsic characteristics of the hair follicle.
• There are various types of hair which include:
  • Lanugo hair: The soft hair which covers the fetal skin and it disappears within the first three months after the fetus delivery.
  • Vellus hair: This is fine, soft hair that is not pigmented. It covers most of the body before the pubertal period.
  • Terminal hair: This is long, coarse and pigmented. Pubertal androgens for example dihydrotestosterone (DHT) convert vellus hair to terminal hair.

• There are 3 phases of hair growth cycle:
  • The hair growth phase (termed Anagen phase): This phase varies, depending on the body area affected. It is approximately 4 months for facial hair.
  • The involutional phase (Catagen phase): This phase is approximately 2-3 weeks.
  • The resting phase (Telogen phase): The hair shaft separates from the dermal papillae at the base of the follicle, which terminates growth.

• The growth of sexual hair is dependent on the presence of androgens.
• Androgens induce vellus follicles in sex-specific areas to develop into terminal hairs, which are larger and more heavily pigmented.
• Hirsutism is caused by increased androgen production and/or an increased sensitivity of the hair follicles to androgens.
• Hyperandrogenism, resulting from any factors, prolongs the anagen(growth) phase of androgen-sensitive hairs, resulting in their conversion from fine, light, vellus hairs to coarse, dark, terminal hairs.
• The response of hair follicle to androgens and other factors such as local 5 alpha-reductase activity determines the level of conversion of hair from the vellus type to terminal hair.^[1]

• Growth recommences with the formation of new hair shaft by the reactivation of the dermal papillae, thereby replacing the old hair.
• The hair growth cycle takes months to years to be completed, causing a delay in hair growth response to changes from effects of androgens.
• DHT is a hormone that acts on the hair follicle to produce terminal hair.
• Differences in the activity of DHT explains why women with the same plasma level testosterone, have different degrees of hirsutism.
• It is speculated that insulin, at high enough concentration, stimulates the ovarian theca cells to produce androgens.
• There may also be an effect of high levels of insulin to activate the insulin-like growth factor-I (IGF-1) receptor in those same cells resulting in increased androgen production.

Common conditions associated with hirsutism include:

• Diabetes (in PCOS )
• Acne
• Fertility problems

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: ; Ogheneochuko Ajari, MB.BS, MS [2] Rasam Hajiannasab M.D.[3]

The cause of hirsutism can be either an increased level of androgens (male hormones) or an oversensitivity of hair follicles to androgens, and the most common cause is polycystic ovary syndrome. Male hormones such as testosterone stimulate hair growth, increase size and intensify the pigmentation of hair. Other symptoms associated with a high level of male hormones include acne and deepening of the voice and increased muscle mass. Growing evidence implicates high circulating levels of insulin in women to the development of hirsutism. This theory is consistent with the observation that obese (and thus presumably insulin resistant hyperinsulinemic) women are at high risk of becoming hirsute. Further, treatments that lower insulin levels will lead to a reduction in hirsutism.

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

• There are no known life threatening causes of hirsutism.

• Androgen secreting tumors ^[1]
• Congenital adrenal hyperplasia
• Cushing’s syndrome
• Idiopathic hirsutism
• Polycystic ovary syndrome ^[2]

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ogheneochuko Ajari, MB.BS, MS [2], Rasam Hajiannasab M.D.[3], Ahmed Elsaiey, MBBCH [4]

Hirsutism must be differentiated based on the different diseases causing hirsutism such as 21-hydroxylase deficiency, 11-β hydroxylase deficiency, 3 beta-hydroxysteroid dehydrogenase deficiency, polycystic ovary syndrome, Cushing’s syndrome, and hyperprolactinemia.

• The diagnosis of hirsutism requires the exclusion of related disorders with peripheral androgen activity.
• Hirsutism starts shortly after puberty with a slow course and progression.
• Hirsutism must be differentiated from other diseases causing excessive hair growth on the body such as 21-hydroxylase deficiency, 11-β hydroxylase deficiency, 3 beta-hydroxysteroid dehydrogenase deficiency, polycystic ovary syndrome, Cushing’s syndrome, and hyperprolactinemia.^[1][2][3]

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ogheneochuko Ajari, MB.BS, MS [2], Rasam Hajiannasab M.D.[3], Ahmed Elsaiey, MBBCH [4]

Hirsutism only affects women, since the rising of androgens causes a male pattern of body hair, particularly in locations where women normally do not develop terminal hair within their puberty (chest, abdomen, back and face). The medical term for excessive hair growth that affect both men and women is hypertrichosis.

• In the United States, the prevalence of hirsutism is about 10,000 per 100,000 individual.^[1][2]
• In the United States, there are at least 4 million premenopausal women who suffer from hirsutism.^[3]

• Hirsutism is more prevalent in the Mediterranean, Middle Eastern, European, and South Asian races.^[4]
• Hirsutism usually affects Hispanic women with polycystic ovarian cysts more than the non-Hispanic women.^[5]
• There is no difference in the prevalence of hirsutism between the white and black races.^[6][2]

• Hirsutism is more prevalent in women, and it is mainly described as a phenomenon in women rather than men.
• Early growth of hair in the pre-pubertal children is an indicator of precocious puberty and it may be a sign of an underlying medical condition.

• Prevalence of hirsutism increases during puberty ages.

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: ; Ogheneochuko Ajari, MB.BS, MS [2] Rasam Hajiannasab M.D.[3]

Common risk factors in the development of hirsutism include ethnicity, particularly women of Mediterranean (Southern European, Middle Eastern), Nordic, East Indian, and Asian ancestry, obesity, and hyperinsulinemia.

Common risk factors in the development of hirsutism include:^[1]

• Familial hirsutism
  • More common in certain ethnic groups:
    • Mediterranean (Southern European, Middle Eastern)
    • Nordic
    • East Indian
    • Asian ancestry
• Obesity
  • May cause insulin resistance
  • May cause reduction in sex hormone-binding globulin
• Hyperinsulinemia
  • May trigger excess ovarian androgen production and decreased sex hormone-binding globulin

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Rasam Hajiannasab M.D.[2]

According to the United States Preventive Services Task (USPSTF), there is insufficient evidence to recommend routine screening for hirsutism.

According to the United States Preventive Services Task (USPSTF), there is insufficient evidence to recommend routine screening for hirsutism.

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]: Associate Editor(s)-in-Chief: ; Ogheneochuko Ajari, MB.BS, MS [2]; Rasam Hajiannasab M.D.[3]

Pubertal onset hirsutism specially when it is mild ,points toward PCOS or idiopathic hirsutism but sever late onset hirsutim with other virlization signs can be due to ovarian or adrenal tumors. Hirsutism can lead to significant psychological distress for women and even depression if left untreated and based on the underlying cause other medical complications can occur (e.g. in cases of hirsutism due to PCOS , if the treatment doesn’t address PCOS , it can lead to infertility.) Prognosis depend on underlying etiology .

The patient’s age of onset of hirsutism can be helpful in the etiologic diagnosis.

• Adrenarche and puberty
  • Early development of pubic hair points toward CAH, because the development of pubic hair depends on adrenal androgens
  • In patients with ovarian hyperandrogenism normal adrenarche and delayed menarche or irregular menses would be expected.^[1]

Hirsutism sometimes can lead to significant psychological distress in patients.^[2] As well as the physical signs of excess hair, hirsutism can affect patients psychologically (mentally). It can cause embarrassment and affect patient’s self-confidence, and therefore the quality of life.Some women feel self-conscious about having unwanted body hair or even may develop depression. Also, although hirsutism doesn’t cause physical complications, the underlying cause of a hormonal imbalance can. For example if hirsutism is due to PCOS , it can lead to infertility.

Prognosis depends on the underlying cause.

Pubertal onset hirsutism, mild hirsutism with slow progression are usually due to benign causes .

In cases of late onset hirsutism (in older women) , rapid and severe hirsutism with other signs of virilization ( including frontal balding, clitoromegaly, increased muscle mass, or deepening of the voice)  , adrenal or ovarian tumors should be considered.^[1]

Template:WH Template:WS