A Rare Case of Adolescent Girl with Beard-PCOS with a Difference: “HAIR-AN Syndrome”

Abstract

“Hyperandrogenism” is one of the commonest reasons for consultation in the adolescent age group. The most common reason being polycystic ovarian syndrome (PCOS) caused due to the changing lifestyle and adopting to a more sedentary type of living. PCOS is an endocrine disorder becoming increasingly common in adolescent, with an incidence of 6–18% in the female population. HAIR-AN syndrome being a specific subtype of PCOS consists of hyperandrogenism (HA), insulin resistance (IR) and acanthosis nigricans (AN) with adipose tissue dysfunction as the initial pathophysiological stimulant. We report a 16-year-old developmentally normal adolescent girl with excessive facial hair growth, an irregular menstrual cycle, and hyperpigmentation who attained menarche at the age of 12. On clinical examination, she had features suggestive of HA and IR. Biochemical investigations showed highly elevated serum insulin with elevated testosterone and normal blood glucose.A multidisciplinary approach with lifestyle modifications combined with hormonal medications like oral contraceptive pills with metformin and spironolactone were required to control the disease and prevent the systemic manifestation and decrease the degree of virilization. As HAIR-AN syndrome is a disorder characterized by high IR, pediatricians, adolescent specialists, and obstetricians should be familiar with this condition in order to assess and manage it efficiently.

Keywords

• hair-an syndrome
• hyperandrogenism
• acanthosis nigricans
• insulin resistance