HAIR-AN Syndrome: A Rare Cause of Hyper Insulinemic State

Sriram Mudraje; Nancy Garg; Jaideep Khare; Sushil Jindal

doi:10.18502/crcp.v9i6.18947

Sriram Mudraje Department of Endocrinology, People’s College of Medical Sciences and Research Centre, People’s University, Bhopal, India.
Nancy Garg Department of Endocrinology, People’s College of Medical Sciences and Research Centre, People’s University, Bhopal, India.
Jaideep Khare Department of Endocrinology, People’s College of Medical Sciences and Research Centre, People’s University, Bhopal, India.
Sushil Jindal Department of Endocrinology, People’s College of Medical Sciences and Research Centre, People’s University, Bhopal, India.

DOI: https://doi.org/10.18502/crcp.v9i6.18947

Keywords: HAIR-AN syndrome; Hyperinsulinemia; Insulin resistance; Hyperandrogenemia

Abstract

Insulin resistance is categorized as type A or type B, depending on the etiology. HAIRAN syndrome is a subtype of type A insulin resistance. It is characterized by high insulin resistance, hyperandrogenemia, and hyperinsulinemia. Here, we present a 12-year-old girl who was lean during her first decade of life and came with complaints of gradual-onset weight gain, darkening of her neck and limbs, and roughening of her skin. Menarche had not been attained. She had Grade 4 acanthosis nigricans, thick coarse skin, and hirsutism. Investigations revealed raised blood glucose, elevated testosterone, elevated fasting insulin, and raised HOMA-IR. With biochemical evidence of hyperandrogenemia, insulin resistance, and the presence of acanthosis nigricans, the diagnosis of HAIR-AN syndrome was made.