Hirsutism

File:PMC4103002 ircmj-16-9410-g001.png

Hirsutism depicted in a female patient with PCOS and nonclassic congenital adrenal hyperplasia

Hirsutism is excessive body hair on parts of the body where hair is normally absent or minimal. The word is from early 17th century: from Latin hirsutus meaning "hairy".<ref>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> It usually refers to a male pattern of hair growth in a female that may be a sign of a more serious medical condition,<ref name=merck>{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> especially if it develops well after puberty.<ref>Template:Cite journal</ref> Cultural stigma against hirsutism can cause much psychological distress and social difficulty.<ref>Template:Cite journal</ref> Discrimination based on facial hirsutism often leads to the avoidance of social situations and to symptoms of anxiety and depression.<ref name="Jackson J, Caro JJ, Caro G, Garfield F, Huber F, Zhou W, Lin CS, Shander D, Schrode K, and the Eflornithine HCl Study Group">Template:Cite journal</ref>

Hirsutism is usually the result of an underlying endocrine imbalance, which may be adrenal, ovarian, or central.<ref name=hair>Template:Cite journal</ref> It can be caused by increased levels of androgen hormones. The amount and location of the hair is measured by a Ferriman–Gallwey score. It is different from hypertrichosis, which is excessive hair growth anywhere on the body.<ref name=merck/>

Treatments may include certain birth control pills, antiandrogens, or insulin sensitizers.<ref name="Barr2018">Template:Cite journal</ref>

Hirsutism affects between 5 and 15% of women across all ethnic backgrounds.<ref>Template:Cite journal</ref> Depending on the definition and the underlying data, approximately 40% of women have some degree of facial hair.<ref name="Blume">Template:Cite journal</ref> About 10 to 15% of cases of hirsutism are idiopathic with no known cause.<ref name="pmid35292252" />

Causes

The causes of hirsutism can be divided into endocrine imbalances and non-endocrine etiologies. It is important to begin by first determining the distribution of body hair growth. If hair growth follows a male distribution, it could indicate the presence of increased androgens or hyperandrogenism. However, there are other hormones not related to androgens that can lead to hirsutism. A detailed history is taken by a provider in search of possible causes for hyperandrogenism or other non-endocrine-related causes. If the distribution of hair growth occurs throughout the body, this is referred to as hypertrichosis, not hirsutism.<ref>Template:Cite journal</ref>

Endocrine causes

Endocrine causes of hirsutism include:

• Ovarian cysts such as in polycystic ovary syndrome (PCOS), the most common cause in women.<ref name="pmid18844715">Template:Cite journal</ref>
• Adrenal gland tumors, adrenocortical adenomas, and adrenocortical carcinoma, as well as adrenal hyperplasia due to pituitary adenomas (as in Cushing's disease).<ref name="pmid21623779" />
• Inborn errors of steroid metabolism such as in congenital adrenal hyperplasia, most commonly caused by 21-hydroxylase deficiency.<ref name="pmid21623779">Template:Cite journal</ref>
• Acromegaly and gigantism (growth hormone and IGF-1 excess), usually due to pituitary tumors.<ref name="pmid21623779" />

Non-endocrine causes

Causes of hirsutism not related to hyperandrogenism include:

• Familial: Family history of hirsutism with normal androgen levels.<ref name=":3">Template:Cite journal</ref>
• Drug-induced: medications were used before the onset of hirsutism. The recommendation is to stop the medication and replace it with another.<ref name=":0" />
  • Minoxidil<ref name="pmid26903750">Template:Cite journal</ref><ref name="pmid12702063">Template:Cite journal</ref>
  • Androgens like testosterone, anabolic steroids, and androgenic progestins<ref name=":3" /><ref name=":0" />
• Valproic acid and methyldopa<ref name=":3" /><ref name=":0" />
• Pregnancy: Due to changes in hormone production<ref name=":6" />
• Idiopathic: When no other cause can be attributed to an individual's hirsutism, the cause is considered idiopathic by exclusion.<ref name=":0" /> In these cases, menstrual cycles and levels of conventionally tested androgens (testosterone, androstenedione, and dehydroepiandrosterone sulfate) are normal.<ref name=":4">Template:Cite journal</ref> Around 10 to 15% of women with hirsutism have idiopathic hirsutism.<ref name="pmid35292252">Template:Cite journal</ref> Idiopathic hirsutism may be due to increased production of dihydrotestosterone (DHT) in hair follicles and hence may actually still be due to hyperandrogenism.<ref name="pmid35292252" /> It may be detectable by measurement of DHT or DHT metabolites.<ref name="pmid35292252" />
  • Rice et al. 2016 propose that idiopathic hirsutism is caused by epigenetic inheritance of discordant epigenetic markers. It is testable with current technology.<ref>Template:Cite journal</ref>

Hormonal causes:<ref name=":3" />	Description:	Clinical cues:
Polycystic ovary syndrome	PCOS is a condition characterized by excess androgens that can lead to hirsutism, irregular periods, and even infertility. The excess androgens can lead to disruptions in normal body hormones in the hypothalamic-pituitary-gonadal axis leading to these symptoms.<ref name="pmid31384717">Template:Cite journal</ref> With PCOS, hair may grow on the face (like on the upper lip, chin, or jawline), chest, stomach, and back.<ref>{{#invoke:citation/CS1|citation	CitationClass=web }}</ref>	Characterized by having two of three Rotterdam criteria: Oligomenorrhea (fewer than eight menses in a year) Clinical or biochemical evidence of hyperandrogenism Polycystic ovaries on ultrasound <ref>Template:Cite journal</ref>
Cushing's syndrome	Cushing syndrome occurs when there is an endogenous or exogenous elevated levels of cortisol. One cause of endogenous Cushing syndrome is an adrenocorticotrophic hormone-secreting pituitary adenoma that is responsible for high secretion of not just cortisol but also androgens from the pituitary gland.<ref name=":5">Template:Cite journal</ref>	Cushing syndrome has an apparent symptoms including: Hirsutism weight gain, extra fat build up around the face, abdominal striae, and irregular menstruation.<ref name=":5" />
Congenital adrenal hyperplasia	CAH can be attributed to several enzymatic deficiencies but the most common is 21-beta-hydroxylase. In CAH, a missing enzyme responsible for normal cortisol synthesis creates a build-up of androgen precursors. This precursor gets shunted to the androgen synthesis pathway leading to increased levels of androgen. Classical CAH is discovered at birth due to increased androgens during development causing ambiguous genitalia. Meanwhile, non-classical CAH is found in puberty presenting as anovulation.<ref name=":4" />	Can present similar to PCOS in non-classical CAH. Increase levels of 17-hydroxyprogesterone.<ref name=":3" /><ref name=":4" />
Androgen-secreting tumors	Tumors in the adrenal glands or in the ovaries leading to increase levels of androgens.<ref name=":4" />	Rapid progression and virilization symptoms.<ref name=":3" />
Other less common hormonal causes:	Acromegaly: Elevated levels of insulin-like growth factor-1.<ref name=":6">Template:Citation</ref> Hyperthyroidism or hypothyroidism: Elevated or decreased levels of thyroid hormones.<ref name=":6" /> Hyperprolactinemia: Elevated levels of prolactin.<ref name=":6" />	Each of these have their own distinct presentation.<ref name=":4" />

Diagnosis

Hirsutism is a clinical diagnosis of excessive androgenic, terminal hair growth.<ref>Template:Cite journal</ref> A complete physical evaluation should be done prior to initiating more extensive studies, the examiner should differentiate between widespread body hair increase and male pattern virilization.<ref name=":0">Template:Cite journal</ref> One method of evaluating hirsutism is the Ferriman-Gallwey Score which gives a score based on the amount and location of hair growth.<ref name="pmid13892577">Template:Cite journal</ref> The Ferriman-Gallwey Score has various cutoffs due to variable expressivity of hair growth based on ethnic background.<ref>Template:Cite journal</ref><ref>Template:Cite journal</ref>

Diagnosis of patients with even mild hirsutism should include assessment of ovulation and ovarian ultrasound, due to the high prevalence of polycystic ovary syndrome (PCOS), as well as 17α-hydroxyprogesterone (because of the possibility of finding non-classic 21-hydroxylase deficiency<ref name="pmid19338993">Template:Cite journal</ref>). People with hirsutism may present with an elevated serum dehydroepiandrosterone sulfate (DHEA-S) level, however, additional imaging is required to discriminate between malignant and benign etiologies of adrenal hyperandrogenism.<ref>Template:Cite journal</ref> Levels greater than 700 μg/dL are indicative of adrenal gland dysfunction, particularly congenital adrenal hyperplasia due to 21-hydroxylase deficiency. However, PCOS and idiopathic hirsutism make up 90% of cases.<ref name=":0"/>

Treatment

Treatment of hirsutism is indicated when hair growth causes patient distress. The two main approaches to treatment are pharmacologic therapies targeting androgen production/action, and direct hair removal methods including electrolysis and photo-epilation. These may be used independently or in combination.<ref>Template:Cite journal</ref>

Pharmacologic therapies

Common medications consist of antiandrogens, insulin sensitizers, and oral contraceptive pills. All three types of therapy have demonstrated efficacy on their own, however insulin sensitizers are shown to be less effective than antiandrogens and oral contraceptive pills.<ref name=":2">Template:Cite journal</ref> The therapies may be combined, as directed by a physician, in line with the patient's medical goals. Antiandrogens are drugs that block the effects of androgens like testosterone and dihydrotestosterone (DHT) in the body.<ref name="pmid21623779" /> They are the most effective pharmacologic treatment for patient-important hirsutism, however they have teratogenic potential, and are therefore not recommended in people who are pregnant or desire pregnancy. Current data does not favor any one type of oral contraceptive over another.<ref name=":2" />

List of medications:

• Bicalutamide: A pure antiandrogen.<ref name="WilliamsBigby2009">Template:Cite book</ref><ref name="pmid24455796">Template:Cite journal</ref><ref name="pmid11915584">Template:Cite journal</ref> It is effective similarly to flutamide but is much safer as well as better-tolerated.<ref name="WilliamsBigby2009" /><ref name="pmid24455796" /><ref name="pmid11915584" />
• Birth control pills that consist of an estrogen, usually ethinylestradiol, and a progestin are supported by the evidence.<ref name=":1" /><ref name="Barr2018" /> They are functional antiandrogens. In addition, certain birth control pills contain a progestin that also has antiandrogenic activity.<ref name="Ekback2017">Template:Cite journal</ref> Examples include birth control pills containing cyproterone acetate, chlormadinone acetate, drospirenone, and dienogest.<ref name="Ekback2017" /><ref name="Blume-PeytaviWhiting2008" />
• Cyproterone acetate: A dual antiandrogen and progestogen.<ref name="pmid24889738" /> In addition to single form, it is also available in some formulations of combined oral contraceptives at a low dosage (see below).<ref name="pmid24889738" /> It has a risk of liver damage.
• Eflornithine: Blocks putrescine that is necessary for the growth of hair follicles.<ref>Template:Cite journal</ref>
• Finasteride and dutasteride: 5α-Reductase inhibitors.<ref name="Blume-PeytaviWhiting2008" /> They inhibit the production of the potent androgen DHT.<ref name="Blume-PeytaviWhiting2008" /> A meta-analysis showed inconsistent results of finasteride in the treatment of hirsutism.<ref name=":1" />
• Flutamide: A pure antiandrogen.<ref name="pmid24889738" /> It has been found to possess equivalent or greater effectiveness than spironolactone, cyproterone acetate, and finasteride in the treatment of hirsutism.<ref name="Publishers1999">Template:Cite book</ref><ref name="pmid24889738" /> However, it has a high risk of liver damage and hence is no longer recommended as a first- or second-line treatment.<ref name="pmid28379593">Template:Cite journal</ref><ref name="Ostrzenski2002">Template:Cite book</ref><ref name="Blume-PeytaviWhiting2008">Template:Cite book</ref><ref name="Becker2001">Template:Cite book</ref> Flutamide is safe and effective.<ref name=":1">Template:Cite journal</ref>
• GnRH analogues: Suppress androgen production by the gonads and reduce androgen concentrations to castrate levels.<ref>Template:Cite journal</ref>
• Metformin: Insulin sensitizer. Antihyperglycemic drug used for diabetes mellitus and treatment of hirsutism associated with insulin resistance (e.g. polycystic ovary syndrome). Metformin appears ineffective in the treatment of hirsutism, although the evidence was of low quality.<ref name=":1" />
• Spironolactone: An antimineralocorticoid with additional antiandrogenic activity at high dosages.<ref name="pmid18389188">Template:Cite journal</ref><ref name="pmid24889738">Template:Cite journal</ref>

Other methods

• Electrology
• Epilation
• Laser hair removal
• Lifestyle change, including reducing excessive weight and addressing insulin resistance, may be beneficial. Insulin resistance can cause excessive testosterone levels in women, resulting in hirsutism.<ref>Template:Cite journal</ref>
• Shaving
• Waxing

See also

• Androgenic hair
• Bearded lady
• Ferriman-Gallwey score
• Hair removal
• Hypertrichosis
• Laser hair removal
• Petrus Gonsalvus
• Polycystic ovary syndrome (PCOS)
• Pubic hair
• Social model of disability
• Trichophilia

External links

• Template:Wiktionary-inline
• Template:Commons category-inline

• Why the Bearded Lady Was Never a Laughing Matter: Hirsutism
• The Bearded Lady