Hyperandrogenism-insulin resistance-acanthosis nigricans (HAIR-AN) syndrome is a special and rare subtype of polycystic ovarian syndrome. It can lead to hyperandrogenism (HA), insulin resistance (IR), and acanthosis nigricans (AN) accompanied by acne, hirutism, irregular menstruation, and other androgen excess symptoms. A case of pediatric HAIR-AN syndrome with severe AN was admitted to the Department of Endocrinology, China-Japan Friendship Hospital. The patient's clinical manifestations, laboratory data, imaging features, and gene sequencing were analyzed, and the patient was diagnosed with pediatric HAIR-AN syndrome. Obesity, IR, hyperglycemia, menstrual disorder, and AN were significantly improved after treating with metformin and liraglutide. HAIR-AN syndrome occurs in various forms. When the patient appears unexplained acanthosis nigricans and menstrual disorders, the disease should be considered possible. Early diagnosis and symptomatic supportive treatment can improve the quality of life.
高雄激素血症-胰岛素抵抗-黑棘皮病(hyperandrogenism-insulin resistance-acanthosis nigricans，HAIR-AN)综合征是多囊卵巢综合征的一种特殊且罕见亚型，可导致患者出现高雄激素血症(hyperandrogenism，HA)、胰岛素抵抗(insulin resistance，IR)和黑棘皮病(acanthosis nigricans，AN)的同时伴有痤疮、多毛症、月经不调等雄激素过多的症状。中日友好医院内分泌科收治1例以严重AN为表现的HAIR-AN综合征患者，对患者的临床表现、实验室资料、影像学特征、基因测序进行分析，明确诊断为儿童HAIR-AN综合征。予二甲双胍联合利拉鲁肽治疗后，患者的肥胖、IR、高血糖、月经不调、AN等情况均得到明显改善。HAIR-AN综合征的发病形式多样，当患者出现不明原因的黑棘皮及月经不调等表现，应考虑该病的可能。早期诊断和对症支持治疗可提高患者的生存质量。.
Keywords: acanthosis nigricans; hyperandrogenism; irregular menstruation; obesity; severe insulin resistance.