Clinical Profiles, Occurrence, and Management of Adolescent Patients with HAIR-AN Syndrome

Abstract

The syndrome of hyperandrogenism, insulin resistance, and acanthosis nigricans (HAIR-AN) is a subphenotype of the polycystic ovary syndrome. It is one of the most common causes of menstrual problems, hyperandrogenic symptoms, and insulin resistance among young women. Review of clinical data in an outpatient adolescent clinic showed that of the 1,002 young women (ages 10-21 years) attending the clinic over a 2-year period, 50 (5%) were diagnosed with HAIR-AN syndrome. Mean age of the patients was 15.5, initial mean weight at diagnosis was 94.5 kg, and the mean BMI was 33.33 kg/m 2 . Patients were treated with a weight-stabilization and -reduction program, oral contraceptive pills, and in most cases metformin. 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. 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.

Acanthosis Nigricans during an HAIR-AN syndrome: "The Tree That Hides the Forest"

“…Apart from virilization, other signs of hyperandrogenism include menstrual disorders such as amenorrhea, hypofertility or sterility, retentional acne, and androgenetic alopecia. If the patient has polycystic ovaries together with signs of hyperandrogenism, the syndrome of polycystic ovary is concluded [3,5,7,10,12]. Patients with HAIR-AN syndrome usually have a high serum level of testosterone, A normal urine level of 17-ketosteroids, 17-hydroxyprogesterone, dihydroepiandrostenedione (DHEA) and normal serum level of basal gonadotrophins (LH, FSH).…”

“…Hyperinsulinemia and associated hyperandrogenism will cause other metabolic, gynecological and psychiatric comorbidities. [1,2,4,5,9,10].…”

“…Patients with HAIR-AN syndrome usually have a high serum level of testosterone, A normal urine level of 17-ketosteroids, 17-hydroxyprogesterone, dihydroepiandrostenedione (DHEA) and normal serum level of basal gonadotrophins (LH, FSH). Normal blood ionogram and cortisiluria make it possible to reject Cushing's syndrome [2,5,7,10,12].…”

“…In some cases, there may be psychiatric disorders of organic origin. The aesthetic damage caused by skin lesions, combined with functional damage from systemic hurts, often leads to real psychological disturbances [10,13,14].…”

“…Its prevalence may be underestimated because of its often insidious manifestations. It is a rare condition which is characterized by an unusual multisystem disorder in women with many cases remaining undiagnosed [1,5,[7][8][9][10][11].…”