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1例伴库欣综合征及肾上腺功能不全的黑棘皮病,病因是长期外用类固醇激素并文献复习

A case of Hailey-Hailey disease accompanied by Cushing's syndrome and adrenal insufficiency due to long-term usage of topical steroids with review of literature.

作者信息

Iwamoto Yuichiro, Komi Masahiro, Fushimi Yoshiro, Kimura Tomohiko, Shimoda Masashi, Nakanishi Shuhei, Aoyama Yumi, Mune Tomoatsu, Kaneto Hideaki

机构信息

Department of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School, Okayama 701-0192, Japan.

Department of Dermatology, Kawasaki Medical School, Okayama 701-0192, Japan.

出版信息

Endocr J. 2025 Aug 1. doi: 10.1507/endocrj.EJ25-0164.

DOI:10.1507/endocrj.EJ25-0164
PMID:40754446
Abstract

Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is a rare autosomal dominant disorder characterized by recurrent vesicles and erosions in intertriginous areas. Topical corticosteroids are the primary treatment, but their potential systemic side effects are often overlooked. Prolonged use on compromised skin can lead to excessive absorption, increasing the risk of iatrogenic Cushing's syndrome and adrenal insufficiency. Here, we report the case of a 50-year-old woman with HHD who had been using topical clobetasol or betamethasone for over 10 years, reaching doses up to 50 g/day. She developed Cushingoid features, metabolic abnormalities, and suppression of the hypothalamic-pituitary-adrenal (HPA) axis. After tapering off topical corticosteroids, she developed adrenal insufficiency and associated withdrawal symptoms. Following the initiation of hydrocortisone replacement therapy, psychiatric symptoms, impaired glucose tolerance, and osteoporotic fractures emerged, suggesting exacerbation of iatrogenic Cushing's syndrome. This case highlights the risk of systemic complications from chronic topical corticosteroid use, particularly in high-absorption areas. Gradual dose reduction, close endocrine monitoring, and individualized tapering strategies are essential to prevent severe outcomes. Clinicians should be aware of potential adrenal suppression and consider endocrine evaluation in patients receiving prolonged, high-dose topical corticosteroid therapy.

摘要

黑利-黑利病(HHD),即家族性良性慢性天疱疮,是一种罕见的常染色体显性疾病,其特征为在皮肤褶皱部位反复出现水疱和糜烂。外用皮质类固醇是主要治疗方法,但其潜在的全身副作用常常被忽视。在受损皮肤上长期使用可能导致药物过度吸收,增加医源性库欣综合征和肾上腺功能不全的风险。在此,我们报告一例50岁患有HHD的女性病例,她使用外用氯倍他索或倍他米松超过10年,剂量高达每天50克。她出现了库欣样特征、代谢异常以及下丘脑-垂体-肾上腺(HPA)轴受抑制。在逐渐减少外用皮质类固醇剂量后,她出现了肾上腺功能不全及相关的戒断症状。在开始氢化可的松替代治疗后,出现了精神症状、糖耐量受损和骨质疏松性骨折,提示医源性库欣综合征加重。该病例突出了长期外用皮质类固醇使用导致全身并发症的风险,尤其是在高吸收部位。逐渐减少剂量、密切的内分泌监测以及个体化的减量策略对于预防严重后果至关重要。临床医生应意识到潜在的肾上腺抑制,并在接受长期、高剂量外用皮质类固醇治疗的患者中考虑进行内分泌评估。

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