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).
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 . 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 .
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 .
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.
Entire Body System
- Weight Gain
15-year-old adolescent girl presented with weight gain, irregular menstrual cycle, skin lesions suggestive of acanthosis nigricans and hirsutism. [semanticscholar.org]
Overall, Cushing's syndrome is a very rare cause of weight gain. In other words, most weight gain that occurs in the United States is a result of diet and exercise behaviors, and not Cushing's syndrome. [endocrinediseases.org]
Along with irregular periods, the first signs of PCOS may be the growth of facial and male-patterned body hair, thinning scalp hair, acne, and weight gain. Weight gain, however, is not always present. Normal-weight women can also have PCOS. [hormone.org]
Symptoms include Irregular menstrual periods Infertility Pelvic pain Excess hair growth on the face, chest, stomach, or thighs Weight gain Acne or oily skin Patches of thickened skin Women with PCOS are at higher risk of diabetes, metabolic syndrome, [medlineplus.gov]
Some common side effects of Diane-35 include light-headedness, fluid retention, weight gain and rare reports of adrenal insufficiency. [obgyn.net]
- 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]
Effect of long-term treatment with metformin added to hypocaloric diet on body composition, fat distribution, and androgen and insulin levels in abdominally obese women with and without the polycystic ovary syndrome. [medscape.com]
A distal hemipancreatectomy with splenectomy was performed in 1988, and her hypoglycaemia ceased. In 1989, she developed torsion of the left ovary, treated by salpingo-oophorectomy. Peri-operative findings excluded polycystic ovaries. [pmj.bmj.com]
Jaw & Teeth
Treatment is aimed at decreasing insulin resistance, regulating ovulation, and decreasing acne, acanthosis nigricans, and hirsutism. [ncbi.nlm.nih.gov]
REPORT OF A CASE A 15-year-old obese Mexican girl requested treatment for hirsutism of 1 year's duration. She dropped out of school because her classmates called her "monkey face" (Figure 1). [jamanetwork.com]
The patient presented here is a 21 year-old woman who has secondary amenorrhea, hirsutism, and hyperpigmented skin changes. The patient was given oral contraceptive and spironolactone. [agris.fao.org]
Conclusions Although a number of treatments are effective in arresting androgenic alopecia, they are not FDA approved for use in women with alopecia, nor in hirsutism. [obgyn.net]
[…] similar nonscarring alopecias. [jamanetwork.com]
Consider the following conditions: Alopecia areata. This is one of the most notable causes of pattern baldness. The symptoms of alopecia areata are thought to be caused by underlying inflammation. [healthline.com]
A biopsy is not required for the diagnosis but will show a non-inflammatory, non-scarring alopecia. [dermatologyadvisor.com]
It is one of the uncommon, yet easily diagnosable causes for noncicatricial alopecia in pediatric population, manifesting mainly as thin, sparse or fine hair. Herein, we report a case of LAHS clinically presenting as diffuse woolly hair, an association [ijpd.in]
Trichotillomania, or compulsive hair-eating, is an impulse-control disorder that can be brought on by stress and leads people to pull out their own hair, explains the NHS. [independent.co.uk]
The success of a cochlear implant is therefore largely dependent on the preservation of auditory neurons, as this ensures optimal transmission of the electrical impulses generated by the implant to the auditory cortex. [pasteur.fr]
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  . 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 . 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 .
- Ovarian Mass
The ovarian mass was subsequently excised. A frozen section done at the time was inconclusive. Paraffin sections did, however, show histological features consistent with a right polycystic ovary and mild hyperthecosis (Figure 1). Figure 1A. [annsaudimed.net]
Sonographic accuracy in the diagnosis of ovarian masses. J Reprod Med. 1990 May. 35(5):491-5. [Medline]. Blake MA, Holalkere NS, Boland GW. Imaging techniques for adrenal lesion characterization. Radiol Clin North Am. 2008 Jan. 46(1):65-78, vi. [medscape.com]
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]
Effect of Treatment with Metformin on Omentin-1, Ghrelin and other Biochemical, Clinical Features in PCOS Patients. [connection.ebscohost.com]
This treatment program is frequently helpful and widely used by endocrinologists in the treatment of alopecia, as well as hirsutism and stubbornly resistant cystic acne. [obgyn.net]
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]
Of note, early diagnosis and management improve the prognosis of the patients (4). According to the literature, hair-thread tourniquet syndrome occurs in boys but some studies have reported this syndrome in girls (8). [numonthly.com]
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]
Understanding this etiologic mechanism can be helpful by early diagnosing and preventing the progression of severe complications such as penile amputation (4). [numonthly.com]
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]
Several etiologies are incriminated but the hyperandrogenism-insulin resistance-acanthosis nigricans syndrome is rare. Observation. A 20-year-old girl, having had a five-year-old secondary amenorrhea. [hindawi.com]
Includes all-new chapters on cytology, immunohistochemistry, flow cytometry, molecular biology, and epidemiology and biostatistics. [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]
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]
Epidemiology In a cross-sectional study conducted in a southwestern practice-based research network (N 1133), AN was found in 17% of children and 21% of adults. 1 In 2 studies, AN was present in 36% of patients with newly diagnosed DM and 39% of children [basicmedicalkey.com]
The proposed pathophysiology of this syndrome is also discussed. [ncbi.nlm.nih.gov]
1987 41 Insulin stimulates androgen accumulation in incubations of human ovarian stroma and theca. 61 Barbieri RL...Ryan KJ 6382082 1984 42 Hyperandrogenism, insulin resistance, and acanthosis nigricans syndrome: a common endocrinopathy with distinct pathophysiologic [malacards.org]
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 - Sabinas brittle hair syndrome Not supplied. Diagnosis - Sabinas brittle hair syndrome Symptoms include brittle hair, mild mental retardation and nail dysplasia. [checkorphan.org]
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]
The absence of clarin-1 leads to a progressive hearing impairment, which could be prevented by gene therapy that targets the sensory hair cells in the inner ear. [pasteur.fr]
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]
- Somani N, Harrison S, Bergfeld WF. The clinical evaluation of hirsutism. Dermatol Ther. 2008 Sep-Oct;21(5):376-91.
- Friedman CI, Richards S, Kim MH. Familial acanthosis nigricans. A longitudinal study. J Reprod Med. 1987 Jul;32(7):531-6.
- Magsino CH, Spencer J. Insulin receptor antibodies and insulin resistance. South Med J. 1999;92:717–9.
- Pinhas-Hamiel O, Zeitler P. Clinical problem-solving. The importance of a name. N Engl J Med. 1999;340:1418–21.
- ACOG technical bulletin. Evaluation and treatment of hirsute. Int J Gynaecol Obstet. June. 49:341-6.
- 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.
- Habif TP. Clinical dermatology. Fifth edition. 2009. Edinburgh; Mosby.
- Gordon CM. Menstrual disorders in adolescents. Excess androgens and the polycystic ovary syndrome. Pediatr Clin North Am. 1999;46:519–43.