Endocrine Abstracts

JOINT1592

Introduction: HAIRAN syndrome (Hyperandrogenism, Insulin resistance, Acanthosis nigricans) is a rare and severe subphenotype of polycystic ovary syndrome (PCOS), marked by hyperandrogenism, insulin resistance, and dermatologic manifestations, including acanthosis nigricans. This syndrome primarily affects young females and presents with symptoms like hirsutism, menstrual irregularities, and obesity. HAIRAN syndrome is distinct from typical PCOS due to the significant severity of insulin resistance and the associated metabolic disturbances. The clinical management of HAIRAN syndrome requires a comprehensive approach involving hormonal regulation, insulin-sensitizing agents, and supportive interventions to address both physical and psychological aspects of the condition.

Case Description: We present a 16-year-old female patient diagnosed with HAIRAN syndrome. The patient has a long history of obesity since childhood and presented with complaints of weight gain, excessive facial and body hair, and menstrual irregularities. She had menarche at age 12, but over the past 9 months, she experienced secondary amenorrhea with no menstruation. Her family history is significant for obesity and type 2 diabetes in her mother, and a sibling diagnosed with PCOS. On physical examination, the patient was found to be hypertensive and had severe acanthosis nigricans on the neck, axillae, and cubital fossae. Anthropometric measurements revealed a BMI of 43.7 kg/m², categorizing her as severely obese, with significant signs of insulin resistance. Laboratory investigations revealed elevated androgen levels, including total testosterone of 55 ng/dl (normal range <48 ng/dl), free testosterone of 85 pmol/l (normal range 10-45 pmol/l), and androstenedione levels of 8 nmol/l (normal range 0.8–6.1 nmol/l). Additionally, a low SHBG level of 13 nmol/l (normal range 40–90 nmol/l) was noted, further confirming the presence of hyperandrogenism and contributing to the diagnosis of HAIRAN syndrome.

Conclusion: This case highlights the clinical complexity of HAIRAN syndrome, emphasizing the importance of early diagnosis and comprehensive management. The patient’s treatment regimen included insulin-sensitizing agents (metformin), anti-androgen therapy (spironolactone), and oral contraceptives (ethinylestradiol and cyproterone) for menstrual regulation and reduction of hirsutism. Amlodipine was prescribed to manage hypertension, and the patient was referred for psychological support to address self-esteem and quality of life concerns. Given the rare and multifactorial nature of HAIRAN syndrome, a multidisciplinary approach involving endocrinologists, gynecologists, dermatologists, and psychologists is essential for optimal patient care. Ongoing follow-up is necessary to monitor the patient’s response to treatment and ensure the proper development of menstrual cycles and overall health.