Case Report

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

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.

[1] Elmer KB, George RM. HAIR-AN syndrome: a multisystem challenge. Am Fam Physician. 2001 Jun 15;63(12):2385-90.
PMID: 11430453.
[2] Agrawal K, Mathur R, Purwar N, Mathur SK, Mathur DK. Hyperandrogenism, Insulin Resistance, and Acanthosis
Nigricans (HAIR-AN) Syndrome Reflects Adipose Tissue Dysfunction (“Adiposopathy” or “Sick Fat”) in Asian Indian
Girls. Dermatology. 2021;237(5):797-805. https://doi.org/10.1159/000512918
[3] Shen Z, Hao F, Wei P. HAIR-AN Syndrome in a Male Adolescent With Concomitant Vitiligo. Arch Dermatol. 2009;145(4):492-494. https://doi.org/10.1001/archdermatol.2009.12
[4] Stuart CA, Gilkison CR, Keenan BS, Nagamani M. Hyperinsulinemia and acanthosis nigricans in African
Americans. J Natl Med Assoc. 1997;89:523-7.
[5] Levin TR, Terrell TR, Stoudemire A. Organic mood disorder associated with the HAIR-AN. J Neuropsychiatry Clin Neurosci. 1992;4:51-4. https://doi.org/10.1176/jnp.4.1.51
[6] Morales-Rosello J. HAIR-AN syndrome and mental disorders [Letter]. J Neuropsychiatry Clin Neurosci. 1995;7:538-9.
https://doi.org/10.1176/jnp.7.4.538
[7] Barbieri RL, Ryan KJ. Hyperandrogenism, insulin resistance, and acanthosis nigricans syndrome: a common endocrinopathy with distinct pathophysiologic features. Am J Obstet
Gynecol. 1983;147:90-101. https://doi.org/10.1016/0002-9378(83)90091-1
[8] Goudas VT, Dumesic DA. Polycystic ovary syndrome. Endocrinol Metab Clin North Am. 1997;26:893-912. https://
doi.org/10.1016/S0889-8529(05)70286-3
[9] Taylor AE. Polycystic ovary syndrome. Endocrinol Metab Clin North Am. 1998;27:877-902. https://doi.org/10.1016/S0889-8529(05)70045-1
[10] Futterweit W Polycystic ovary syndrome: clinical perspectives and management. Obstet Gynecol Surv. 1999;54:403-13. https://doi.org/10.1097/00006254-199906000-00024
[11] Esperanza LE, Fenske NA. Hyperandrogenism, insulin resistance, and acanthosis nigricans (HAIR-AN) syndrome:
spontaneous remission in a 15-year-old girl. J Am Acad Dermatol. 1996;34:892-7. https://doi.org/10.1016/S0190-
9622(96)90074-2
[12] Manc o M, Cas tagne to M, Nanni G, Guidone C, Tondolo V, Greco AV, Gasbarrini G, Mingrone G.
Biliopancreatic diversion as a novel approach to the HAIR-AN syndrome. Obes Surg. 2005 Feb;15(2):286-9. http s://doi.org/10.1381/0960892053268435
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IssueVol 9 No 6 (2024): November-December QRcode
SectionCase Report(s)
DOI https://doi.org/10.18502/crcp.v9i6.18947
Keywords
HAIR-AN syndrome Hyperinsulinemia Insulin resistance Hyperandrogenemia
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How to Cite
1.
Mudraje S, Garg N, Khare J, Jindal S. HAIR-AN Syndrome: A Rare Cause of Hyper Insulinemic State. CRCP. 2025;9(6):272-276.