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阿达木单抗治疗系统性硬化症合并坏疽性脓皮病 1 例:罕见病例报告。

Treatment of systemic sclerosis complicated with pyoderma gangrenosum with adalimumab: A case report of a rare disease.

机构信息

Department of Rheumatology, Qingdao Hiser Hospital Affiliated with Qingdao University (Qingdao Traditional Chinese Medicine Hospital), Qingdao, Shandong Province, China.

Department of Traditional Chinese Medicine Classics, Shandong University of Traditional Chinese Medicine Affiliated Hospital, Jinan, Shandong, China.

出版信息

Int J Immunopathol Pharmacol. 2024 Jan-Dec;38:3946320241300137. doi: 10.1177/03946320241300137.

DOI:10.1177/03946320241300137
PMID:39584543
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11587178/
Abstract

Pyoderma gangrenosum (PG) is a rare noninfectious neutrophilic dermatosis characterized by recurrent, painful ulcers that commonly affect the lower extremities but can also involve other parts of the body. Over half of patients with PG have concomitant systemic immune diseases, with the association of PG with systemic sclerosis (SSc) being extremely rare. Treatment of PG primarily involves local therapy, steroids, and immunosuppressants, with an increasing emphasis on biologic agents. Among these, tumor necrosis factor-alpha (TNF-α) antagonists are considered effective. The patient in this report was an elderly female with a history of systemic sclerosis for many years and initially presented with gangrenous ulcers on the fingertips. After inconclusive conventional treatment, adalimumab was added for 5 weeks, resulting in disease suppression, a reduction in ulcer size, and re-epithelialization of the skin lesions after 6 months.

摘要

坏疽性脓皮病(PG)是一种罕见的非传染性中性粒细胞皮肤病,其特征为反复发作、疼痛的溃疡,通常影响下肢,但也可涉及身体的其他部位。超过一半的 PG 患者同时患有系统性自身免疫性疾病,PG 与系统性硬皮病(SSc)的关联极为罕见。PG 的治疗主要包括局部治疗、类固醇和免疫抑制剂,越来越重视生物制剂。其中,肿瘤坏死因子-α(TNF-α)拮抗剂被认为是有效的。本报告中的患者为一名老年女性,患有系统性硬皮病多年,最初表现为指尖坏疽性溃疡。在常规治疗无效后,加用阿达木单抗治疗 5 周,疾病得到抑制,溃疡缩小,6 个月后皮肤病变重新上皮化。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c9b/11587178/68f2dfd73e59/10.1177_03946320241300137-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c9b/11587178/55bce53fdf69/10.1177_03946320241300137-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c9b/11587178/68f2dfd73e59/10.1177_03946320241300137-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c9b/11587178/55bce53fdf69/10.1177_03946320241300137-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c9b/11587178/68f2dfd73e59/10.1177_03946320241300137-fig2.jpg

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本文引用的文献

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Clin Cosmet Investig Dermatol. 2023 Aug 9;16:2143-2152. doi: 10.2147/CCID.S381490. eCollection 2023.
2
Clinical guidance of pyoderma gangrenosum 2022.2022年坏疽性脓皮病临床指南
J Dermatol. 2023 Sep;50(9):e253-e275. doi: 10.1111/1346-8138.16845. Epub 2023 Jun 13.
3
Clinicopathological features and prognosis of pyoderma gangrenosum in Korea: A single centre, retrospective, observational study over 20 years.
韩国坏疽性脓皮病的临床病理特征及预后:一项单中心、回顾性、20年观察性研究
Indian J Dermatol Venereol Leprol. 2023 Jan-Frebuary;89(1):25-34. doi: 10.25259/IJDVL_968_20.
4
Adalimumab in Japanese patients with active ulcers of pyoderma gangrenosum: Final analysis of a 52-week phase 3 open-label study.阿达木单抗治疗日本脓疱性坏疽性脓皮病活动期溃疡患者:一项 52 周开放标签 3 期研究的最终分析。
J Dermatol. 2022 May;49(5):479-487. doi: 10.1111/1346-8138.16337. Epub 2022 Mar 3.
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Ustekinumab as adjuvant treatment for all pyoderma gangrenosum subtypes.乌司奴单抗作为所有坏疽性脓皮病亚型的辅助治疗。
J Dermatolog Treat. 2022 Jun;33(4):2386-2390. doi: 10.1080/09546634.2021.1937475. Epub 2021 Jun 14.
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Characterizing the presence of neutrophil extracellular traps in neutrophilic dermatoses.描述中性粒细胞皮肤病中中性粒细胞胞外陷阱的存在。
Exp Dermatol. 2021 Jul;30(7):988-994. doi: 10.1111/exd.14360. Epub 2021 May 9.
7
Dermatological Manifestations in Inflammatory Bowel Diseases.炎症性肠病的皮肤表现
J Clin Med. 2021 Jan 19;10(2):364. doi: 10.3390/jcm10020364.
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9
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