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高雄激素血症-胰岛素抵抗-黑棘皮症综合征

Hyperandrogenism-Insulin Resistance-Acanthosis Nigricans Syndrome.

作者信息

Dédjan A H, Chadli A, El Aziz S, Farouqi A

机构信息

Endocrinology, Diabetology and Metabolic Diseases, Ibn Rochd University Hospital Center of Casablanca, 20360 Casablanca, Morocco.

出版信息

Case Rep Endocrinol. 2015;2015:193097. doi: 10.1155/2015/193097. Epub 2015 Jul 2.

DOI:10.1155/2015/193097
PMID:26229697
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4503582/
Abstract

Introduction. Female hyperandrogenism is a frequent motive of consultation. It is revealed by hirsutism, acne or seborrhea, and disorders in menstruation cycle combined or not with virilisation signs. Several etiologies are incriminated but the hyperandrogenism-insulin resistance-acanthosis nigricans syndrome is rare. Observation. A 20-year-old girl, having had a five-year-old secondary amenorrhea. The exam revealed a patient, normotensive with a body mass index at 30 kg/m(2) and a waist measurement of 120 cm, a severe hirsutism assessed to be 29 according to Ferriman Gallwey scale, virilisation signs of male morphotype, clitoridic hypertrophy and frontal alopecia, and an acanthosis nigricans behind the neck, in the armpits and elbows. The assessment carried out revealed testosteronemia at 1.28 ng/mL, which is more than twice the upper norm of the laboratory. Imaging studies were negative for both ovarian and adrenal masses. The retained diagnosis is HAIR-AN syndrome probably related to ovarian hyperthecosis and she was provided with androcur 50 mg/day and estradiol pills 2 mg/day and under hygiene-dietetic conditions. Conclusion. This case proves that HAIR-AN syndrome could be responsible for severe hyperandrogenism with virilisation signs. It must be retained after discarding the tumoral causes and when there are signs of insulin resistance.

摘要

引言。女性高雄激素血症是常见的就诊原因。其表现为多毛、痤疮或脂溢性皮炎，以及月经周期紊乱，可伴有或不伴有男性化体征。病因有多种，但高雄激素血症 - 胰岛素抵抗 - 黑棘皮症综合征较为罕见。

病例报告。一名20岁女性，继发性闭经5年。检查发现该患者血压正常，体重指数为30kg/m²，腰围120cm，多毛严重，根据费里曼 - 盖尔维（Ferriman Gallwey）量表评估为29分，有男性体型的男性化体征、阴蒂肥大和额部脱发，颈部后方、腋窝及肘部有黑棘皮症。检查显示睾酮水平为1.28ng/mL，超过实验室正常上限两倍多。影像学检查未发现卵巢和肾上腺肿物。最终诊断为可能与卵巢卵泡膜细胞增生症相关的高雄激素血症 - 胰岛素抵抗 - 黑棘皮症（HAIR - AN）综合征，并给予患者每天50mg的醋酸环丙孕酮及每天2mg的雌二醇片治疗，同时给予卫生饮食指导。

结论。该病例证明HAIR - AN综合征可能导致伴有男性化体征的严重高雄激素血症。在排除肿瘤性病因且存在胰岛素抵抗迹象时应考虑该诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c5fe/4503582/856c1ae993e5/CRIE2015-193097.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c5fe/4503582/78cc55392ddc/CRIE2015-193097.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c5fe/4503582/56793c994c7a/CRIE2015-193097.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c5fe/4503582/be5e9adcdd38/CRIE2015-193097.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c5fe/4503582/856c1ae993e5/CRIE2015-193097.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c5fe/4503582/78cc55392ddc/CRIE2015-193097.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c5fe/4503582/56793c994c7a/CRIE2015-193097.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c5fe/4503582/be5e9adcdd38/CRIE2015-193097.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c5fe/4503582/856c1ae993e5/CRIE2015-193097.004.jpg

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Severe hyperandrogenemia and insulin resistance in a 12-year-old girl.一名12岁女孩出现严重高雄激素血症和胰岛素抵抗。
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