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HAIR-AN Syndrome

HAIRAN Syndrome

HAIR-AN syndrome is an abbreviation for a rare syndrome, occurring in female patients. It comprises of hyperandrogenism (HA), insulin resistance (IR), and acanthosis nigricans (AN).


Presentation

The symptoms and signs associated with HAIR-AN syndrome are caused by hyperandrogenism, insulin resistance and acanthosis nigricans [1].

Hyperandrogenism tends to present with acne, hirsutism, seborrhea and menstrual abnormalities, such as amenorrhea or infertility. Increased levels of androgens can also induce symptoms including clitoromegaly, androgenic alopecia, an increased muscle mass and a deep voice.

Insulin resistance is a pathological condition, in which the organism's tissues fail to respond to insulin, which is secreted when glucose in released into the bloodstream, due to the catabolization of fats and carbohydrates [2]. Insulin resistance may co-exist with normal or increased levels of serum glucose and is further sub-divided into two categories: type A and B. Type A insulin resistance is a rare inherited condition, caused by genetic mutations in the insulin receptors or varied target cell disorders in insulin response. Type B insulin resistance typically co-exists with less profound manifestations of acanthosis nigricans and is associated with autoantibodies against insulin receptors and antinuclear antibodies [3].

The third manifestation of HAIR-AN syndrome is acanthosis nigricans, a skin pigmentation disorder that encompasses regionalized areas of hyperpigmented skin with a velvety texture. They usually appear mostly on the axilla, neck, and groin and are a result of prolonged exposure of keratinocytes to insulin in patients with HAIR-AN. Acanthosis nigricans is observed in 60-80% of teenagers with diabetes mellitus type 2 [4].

Lastly, HAIR-AN syndrome may also be accompanied by depression, which can often be attributed to hypothalamic irregularities and usually subsides with the administration of contraceptives per os.

Abdominal Obesity
  • Physical examination also can detect high blood pressure and increased abdominal obesity as risk factors for diabetes and cardiovascular disease in individuals who are overweight.[obgyn.ucla.edu]

Workup

The first step towards diagnosing HAIR-AN syndrome includes laboratory tests, such as a complete blood cell count, serum prolactin, glucose and insulin evaluation, thyroid function tests and serum electrolytes. Given the fact that hyperlipidemia and subsequent coronary artery disease are often diagnosed when hyperandrogenism is detected, total cholesterol, high-density lipoprotein (HDL) and low-density lipoprotein (LDL) cholesterol, as well as a triglyceride count should be performed. Additionally, the patient requires testing for antinuclear antibodies and erythrocyte sedimentation rate (ESR), due to the syndrome's frequent association with other autoimmune abnormalities.

Laboratory confirmation of hyperandrogenism is also mandatory, should there be suspicions towards it. Morning cortisol after the administration of a mild dexamethasone dose, dehydroepiandrosterone sulfate (DHEAS) and 17a-hydroxyprogesterone need to be measured, along with testosterone levels. In fact, testosterone measurement is required in order to detect a virilizing tumor, as increased DHEAS is indicative, but not diagnostic of, an androgen-producing tumor of the adrenal glands [5] [6]. Testosterone is usually measured two to three times, for the results to be considered definitive. Lutenizing and follicle-stimulating hormone (LH, FSH) should also be evaluated.

Finally, in cases where acanthosis nigricans is extensive and other cancer-associated signs are observed, meticulous investigation to rule out a potential malignancy should be carried out [7]. An abdominal or pelvic magnetic resonance imaging scan (MRI) or computed tomography scan (CT), along with upper and/or lower endoscopy are some of the modalities that can be employed for detecting a lesion [8].

Treatment

  • Of the patients, 80% were compliant with the follow-up and treatment regimen, 60% maintained or reduced their weight, 95% had regular menstrual cycles, and in most patients, the acne and/or hirsutism were the same or better than at the start of treatment[ncbi.nlm.nih.gov]
  • Treatment is aimed at decreasing insulin resistance, regulating ovulation, and decreasing acne, acanthosis nigricans, and hirsutism.[ncbi.nlm.nih.gov]
  • Although a treatment regimen for the HAIR-AN syndrome has not been established, antiandrogen therapy and weight loss are useful.[ncbi.nlm.nih.gov]
  • Effect of Treatment with Metformin on Omentin-1, Ghrelin and other Biochemical, Clinical Features in PCOS Patients.[connection.ebscohost.com]
  • The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]

Prognosis

  • Prognosis - Sabinas brittle hair syndrome Not supplied. Treatment - Sabinas brittle hair syndrome Encourage gentle hair care to avoid further hair loss. Also, suggest wearing a wig, cap, or scarf, if appropriate.[checkorphan.org]
  • Read: Boy, 10, Spends 2 Years Growing Hair He Donated to Make Wig for Friend, 12, With Alopecia “[There is] no cure for this, but the prognosis of this is really good,” Dr. Bree said.[insideedition.com]
  • Treatment and prognosis It is lethal and the affected males typically die by the time they are aged 2-3 years. A pneumonia or respiratory infection is the usual cause of death. Treatment is mainly supportive.[radiopaedia.org]
  • The prognosis is variable among women with PCOS who undergo fertility treatments. In women who are overweight or obese, ovulation can sometimes be achieved by moderate weight loss alone.[emedicinehealth.com]
  • Newborns with severe symptoms often have a poor prognosis .[dermnetnz.org]

Etiology

  • Identification of the underlying etiology does not alter management, but detects patients at risk for infertility, diabetes, cardiovascular disease and endometrial carcinoma.[ncbi.nlm.nih.gov]
  • For ex ample, we have separated the section devoted to various disease entities into diffuse and circumscribed leukoderma and the latter into various etiologies such as genetic, metabolic, infectious, and endocrinologic.[books.google.it]
  • The etiology is controversial [ 30 ].[intechopen.com]
  • AN can be an adverse effect from hormonal therapies. 7 Etiology and Pathophysiology AN results from of long-term exposure of keratinocytes to insulin.[basicmedicalkey.com]
  • Polycystic Ovary Syndrome (PCOS)ETIOLOGY & PATHOPHYSIOLOGY PCOS 7 8. PCOS: Etiology• Neuroendocrine derangement: LH relative to FSH• Hyperinsulinemia: defect in insulin action or secretion• Androgen excess: ovarian and adrenal PCOS 8 9.[slideshare.net]

Epidemiology

  • Includes all-new chapters on cytology, immunohistochemistry, flow cytometry, molecular biology, and epidemiology and biostatistics.[books.google.com]
  • Our understanding of the epidemiology and economic impact of these disorders has expanded, emphasizing their critical importance. These are but a few highlights of how the terrain has changed in a relatively brief period of time.[books.google.com]
  • Main Outcome Measures Epidemiologic data for all forms of alopecia were ascertained, such as sex, age at onset, age at the time of evaluation, and clinical diagnosis.[jamanetwork.com]
  • The discussion covers the phenotype spectrum, epidemiology, mode of inheritance, pathogenesis, and clinical profile of each condition, all of which is accompanied by a wealth of illustrations.[books.google.it]
  • Epidemiology, diagnosis, and management of polycystic ovary syndrome. Clinical Epidemiology. 2014;6:1-13. doi:10.2147/CLEP.S37559. George, Kathleen. “HAIR-AN Syndrome: A Multisystem Challenge – American Family Physician”.[thehealthsite.com]
Sex distribution
Age distribution

Pathophysiology

  • The proposed pathophysiology of this syndrome is also discussed.[ncbi.nlm.nih.gov]
  • Acanthosis Nigricans Associated with Insulin Resistance: Pathophysiology and Management.[connection.ebscohost.com]
  • "Hyperandrogenism, insulin resistance, and acanthosis nigricans syndrome: A common endocrinopathy with distinct pathophysiologic features. Barbieri RL, Ryan KJ".[en.wikipedia.org]

Prevention

  • We conclude that HAIR-AN syndrome is a common disease in young women and multifaceted, aggressive treatment appears to be effective in reducing the severity of symptoms and preventing further consequences.[ncbi.nlm.nih.gov]
  • Prevention - Sabinas brittle hair syndrome Not supplied. Diagnosis - Sabinas brittle hair syndrome Symptoms include brittle hair, mild mental retardation and nail dysplasia.[checkorphan.org]
  • While it's normal to shed 50-100 hair a day, losing more could show more Did you know sunblock could help prevent skin rashes? Women's Health Skin rashes, no matter how small, can lead to a lot of irritation and be very difficult to deal with.[practo.com]
  • 1997; acanthosis nigricans has virtually cleared Discussion Acanthosis nigricans has been associated with obesity, often with insulin resistance. 1 The resistance to insulin may result in hyperinsulinaemia and a down-regulation of insulin receptors to prevent[pmj.bmj.com]
  • Psychological treatment is recommended to prevent future compulsive hair eating. This is especially important for patients with trichotillomania or stress-related pica because they may be at risk of developing Rapunzel syndrome again .[sbs.com.au]

References

Article

  1. Somani N, Harrison S, Bergfeld WF. The clinical evaluation of hirsutism. Dermatol Ther. 2008 Sep-Oct;21(5):376-91.
  2. Friedman CI, Richards S, Kim MH. Familial acanthosis nigricans. A longitudinal study. J Reprod Med. 1987 Jul;32(7):531-6.
  3. Magsino CH, Spencer J. Insulin receptor antibodies and insulin resistance. South Med J. 1999;92:717–9.
  4. Pinhas-Hamiel O, Zeitler P. Clinical problem-solving. The importance of a name. N Engl J Med. 1999;340:1418–21.
  5. ACOG technical bulletin. Evaluation and treatment of hirsute. Int J Gynaecol Obstet. June. 49:341-6.
  6. Hoffman DI, Klove K, Lobo RA. The prevalence and significance of elevated dehydroepiandrosterone sulfate levels in anovulatory women. Fertil Steril. 1984 Jul.; 42(1):76-81.
  7. Habif TP. Clinical dermatology. Fifth edition. 2009. Edinburgh; Mosby.
  8. Gordon CM. Menstrual disorders in adolescents. Excess androgens and the polycystic ovary syndrome. Pediatr Clin North Am. 1999;46:519–43.

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Last updated: 2018-06-22 08:15