HAIR-AN syndrome

HAIR-AN syndrome consists of hyperandrogenism (HA), insulin resistance (IR) and acanthosis nigricans (AN).[1]: 507 [2] It is a rare disease which is a subset of polycystic ovary syndrome (PCOS).[3] Polycystic ovary syndrome is a condition in females in which there is an increase in the production of androgen (male hormone), leading to an expression of male-like characters in the female body. The name HAIR-AN is made up of the three conditions of which it consists.[4] The commonly used term "HAIR-AN" is a generic description of the features of SIR (severe insulin resistance)."[5] Although HAIR-AN can result from two very different types of abnormalities – blocking antibodies against the insulin receptor or genetically absent/reduced insulin receptor number/function – patients with both types have high levels of androgens (male hormones).

HAIR-AN syndrome
Acanthosis nigricans

Research on the two types of HAIR-AN demonstrated that patients with both forms of HAIR-AN had very high levels of insulin and, critically, that it was the high insulin that caused the elevation in androgen. The patient affected by HAIR-AN syndrome has insulin resistance inside their body. Insulin resistance is a condition in which the body produces insulin but does not use it properly. This causes the pancreas to produce more insulin in the body. High levels of insulin stimulate the ovaries to make excess of androgen, leading to excessive hair growth, acne and irregular periods in the female body. Insulin resistance can also lead to diabetes, heart disease and excessive growth and darkening of the skin (acanthosis nigricans)[6]

Signs and symptoms

In the majority of young women, hyperandrogenism leads to oily skin, acne, hirsutism, menstrual irregularities and, in some cases, androgenic alopecia, clitorimegaly, changes in muscle mass and deepening of the voice. Insulin resistance can be present in different forms; some persons have high concentrations of insulin but normal levels of glucose, while others have glucose measurements in the diabetic range. A history of diabetic symptoms such as polydipsia, polyuria and weight loss may sometimes, but not always, be present.[4] Other related symptoms to HAIR-AN syndrome include enlarged clitoris, increased libido, glucose intolerance, irregular menstruation, increased blood pressure, infertility.[7] Obesity is also one such symptoms in some women, and is also marked in women affected by PCOS, hirsutism, acanthosis nigricans.[4]

Features Manifestations
Hyperandrogenism Hirsutism of the face, chin, chest, perineum
Alopecia (hair loss from the vertex or crown areas of the scalp; bitemporal hair loss less frequent)
Male body habitus (muscularity)
Acne
Clitorimegaly
Menstrual dysfunction (amenorrhea, infertility)
Increased libido
Insulin resistance Polydipsia, polyuria (symptoms of insulin resistance are often subclinical)
Acanthosis nigricans Verrucous, velvety hyperpigmentation on nape of neck, vulva, axillae, groin, umbilicus, submammary regions; increased skin tags
Obesity Increased waist-to-hip ratio (android appearance)

[4]

Causes

Causes of HAIR-AN syndrome are not yet discovered or are not specifically known. But depending on some tests and data collected, HAIR-AN syndrome is thought to be caused by "both genetic and environmental" conditions or factors.[8][3] SAHA syndrome is also taken in consideration among the sources that cause HAIR-An syndrome.[7]

Diagnosis

HAIR-AN syndrome is caused by genetic and environmental factors. It's also caused due to hyperandrogenism, insulin resistance and acanthosis nigricans. One of the ways to diagnose this syndrome is by looking for symptoms that relate to key contributors as discussed above..

According to studies HAIR-AN is to be found in 1% to 3% women possessing hyperandrogenism.[9] It is an established concept in physiopathology that the androgen in the female body is produced by the stromal ovarian cells, when stimulated by the LH and HCG. The observed activity of these cells was elevated by insulin, and later was found to be used as a determining element to find how severe the hirsutism was.[8] Physicians must look for obesity, as it is also a diagnostic factor in many possible cases.

Treatment

HAIR-AN syndrome as discussed earlier is caused by both genetic and environmental factors. It is found that women affected by this syndrome or PCOS (polycystic ovary syndrome) are generally accompanied by obesity. Weight loss is most suggested way to combat this syndrome and is helpful for reducing insulin resistance of the body. It is also a good way to have a control on diet. This might help the body to refunction properly and show some resistance to HAIR-AN syndrome.[8] "Suppression of gonadotropin with estrogen-progesterone oral contraceptives" or can say as reducing hyperandrogenism by the use of estoprogestatif can reduce production of androgen by ovaries by cutting down the LH (leutinizing hormone) level in body.[10][4] Even their sex hormone binding to globulin increase is also responsible for decreasing body's bio-availability of testosterone.[8] There are also a few new progestin pills, such as desogestrel and norgestimate. This pills appear to have fewer androgenic side effects and may be safer to use in persons with abnormal lipid levels or hirsutism.[4] Some antiandrogenic agents can be also used alone or combining it with other oral pills.[11]

"Spironolactone inhibit the actions of testosterone by binding to its receptors." The standard dose for its use is considered to be 50 to 100 mg twice a day.[12] This might lead to irregular menstrual bleeding, which can be improved by oral contraceptives.[4] Flutamide, another antiandorgen that is used to treat HAIR-AN syndrome, but it has risk of hepatotoxicity.[12][4] Finasteride is a 5α-reductase inhibitor which can reduces the conversion of testosterone to dihydrotestosterone. It is useful in the treatment of hirsutism with a dosages as low as 5 mg per day.[13][12][14]

Insulin-resistant patients can also be treated with metformin which has shown promising results to reduce the insulin resistivity.[12][14][15] Metformin improves peripheral tissue sensitivity to insulin but inhibits hepatic glucose formation. The drug reduces the levels of circulating insulin and androgens. Women have shown improved reproductive functioning after the use of metformin.[4]

See also

References
  1. James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  2. Somani N, Harrison S, Bergfeld WF (2008). "The clinical evaluation of hirsutism". Dermatol Ther. 21 (5): 376–91. doi:10.1111/j.1529-8019.2008.00219.x. PMID 18844715.
  3. Rager, K. M.; Omar, H. A. (2006). "Androgen excess disorders in women: the severe insulin-resistant hyperandrogenic syndrome, HAIR-AN". TheScientificWorldJournal. 6: 116–21. doi:10.1100/tsw.2006.23. PMC 5917269. PMID 16435040.
  4. George, Kathleen B. Elmer|Rita M. (2001-06-15). "HAIR-AN Syndrome: A Multisystem Challenge". American Family Physician. 63 (12): 2385–90. PMID 11430453. Retrieved 2017-03-25.
  5. Semple, Robert K.; Williams, Rachel M.; Dunger, David B. (2010). "What is the best management strategy for patients with severe insulin resistance?". Clinical Endocrinology. 73 (3): 286–290. doi:10.1111/j.1365-2265.2010.03810.x. PMID 20455892.
  6. "HAIR-AN syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 2017-03-25.
  7. "HAIR-AN Syndrome Symptoms, Diagnosis, Treatments and Causes - RightDiagnosis.com". rightdiagnosis.com. Retrieved 2017-03-30.
  8. Dédjan, A. H.; Chadli, A.; El Aziz, S.; Farouqi, A. (2015). "Case Report Hyperandrogenism-Insulin Resistance-Acanthosis Nigricans Syndrome". Case Reports in Endocrinology. 2015: 193097. doi:10.1155/2015/193097. PMC 4503582. PMID 26229697.
  9. Azziz, Ricardo; Carmina, Enrico; Dewailly, Didier; Diamanti-Kandarakis, Evanthia; Escobar-Morreale, Héctor F.; Futterweit, Walter; Janssen, Onno E.; Legro, Richard S.; Norman, Robert J. (2009-02-01). "The Androgen Excess and PCOS Society criteria for the polycystic ovary syndrome: the complete task force report". Fertility and Sterility. 91 (2): 456–488. doi:10.1016/j.fertnstert.2008.06.035. ISSN 0015-0282. PMID 18950759.
  10. Barbieri Robert L., Ryan Kenneth J. (1 September 1983). "Hyperandrogenism, insulin resistance, and acanthosis nigricans syndrome: A common endocrinopathy with distinct pathophysiologic features. Barbieri RL, Ryan KJ". American Journal of Obstetrics and Gynecology. 147: Issue 1, 1 September 1983, Pages 90–101. doi:10.1016/0002-9378(83)90091-1.
  11. Goudas, Vasilios T.; Dumesic, Daniel A. (1 December 1997). "POLYCYSTIC OVARY SYNDROME". Endocrinology and Metabolism Clinics of North America. 26 (4): 896–912. doi:10.1016/s0889-8529(05)70286-3.
  12. MD, Scott Moses. "HAIR-AN Syndrome". fpnotebook.com. Retrieved 2017-04-03.
  13. Taylor, Ann E. (1998). "Polycystic ovary syndrom". Endocrinol Metab Clin North Am. 27 (4): 877–902. doi:10.1016/s0889-8529(05)70045-1.
  14. Futterweit, W. (1999). "Polycystic ovary syndrome: clinical perspectives and management". Obstet. Gynecol. Surv. 54 (6): 403–13. doi:10.1097/00006254-199906000-00024. PMID 10358853.
  15. Ehrmann, David A. (1997). "Relation of functional ovarian hyperandrogenism to non-insulin dependent diabetes mellitus". Baillière's Clinical Obstetrics and Gynaecology. 11 (2): 335–47. doi:10.1016/s0950-3552(97)80040-5.
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