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坏疽性脓皮病的一种不寻常表现导致全身炎症反应综合征。

An Unusual Presentation of Pyoderma Gangrenosum Leading to Systemic Inflammatory Response Syndrome.

作者信息

Didan Ali, Donnelly Alan, Chua Hock

机构信息

Department of Dermatology, Fiona Stanley Hospital, Murdoch, Washington, Australia.

出版信息

Case Rep Dermatol. 2017 Sep 11;9(3):146-150. doi: 10.1159/000479923. eCollection 2017 Sep-Dec.

DOI:10.1159/000479923
PMID:29033819
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5636999/
Abstract

This is a report of an atypical presentation of pyoderma gangrenosum (PG) in a 26-year-old male who had a negative septic screen. The patient had a life-threatening presentation requiring an intensive care unit (ICU) admission for vasopressor support. It was thought that the likely cause of circulatory collapse was an overwhelming cytokine reaction or systemic inflammatory response syndrome (SIRS) secondary to extensive PG lesions rather than septic shock. The patient presented with multiple large ulcers, the largest being 4 cm in diameter on the central chest. He developed fevers and circulatory shock preceding his ICU admission. Microbiological specimens, including blood cultures and wound swabs, were negative for any growth (bacterial, fungal, and tuberculosis). No infective foci could be identified as a cause of hemodynamic instability. During admission, the patient's condition was complicated by multi-organ dysfunction. Wound debridement extending to the deep fascia on the anterior chest, back, bilateral shoulders, and right upper thigh was deemed necessary and performed by the plastic surgery team. Histopathology showed abundant neutrophils but could not confirm an infective process. Overall, the patient made an impressive recovery with almost complete healing of all lesions following oral prednisolone alone. Based on the history and clinical and laboratory findings, a diagnosis of PG complicated by a SIRS was favored. Very few cases of neutrophilic dermatoses have been described in this way. A similar presentation has been described in a 76-year-old female with lower-leg ulcers who developed circulatory shock and required an amputation. Lesions continued to appear despite antibiotics and surgical treatment. Septic screen was negative. She was subsequently diagnosed with PG and recovered rapidly after steroid therapy.

摘要

这是一篇关于一名26岁男性坏疽性脓皮病(PG)非典型表现的报告,该患者的败血症筛查结果为阴性。患者出现危及生命的症状,需要入住重症监护病房(ICU)接受血管活性药物支持。据认为,循环衰竭的可能原因是广泛的PG病变继发的压倒性细胞因子反应或全身炎症反应综合征(SIRS),而非感染性休克。患者出现多个大溃疡,最大的位于胸部中央,直径达4厘米。在入住ICU之前,他出现了发热和循环性休克。包括血培养和伤口拭子在内的微生物标本均未发现任何生长(细菌、真菌和结核)。未发现感染灶是血流动力学不稳定的原因。住院期间,患者的病情因多器官功能障碍而复杂化。整形外科团队认为有必要对前胸、背部、双侧肩部和右上大腿进行深筋膜清创术。组织病理学显示有大量中性粒细胞,但无法证实存在感染过程。总体而言,患者仅口服泼尼松龙后恢复良好,所有病变几乎完全愈合。根据病史、临床和实验室检查结果,倾向于诊断为PG合并SIRS。以这种方式描述的嗜中性皮病病例非常少。一名76岁患有小腿溃疡的女性也有类似表现,她出现了循环性休克并需要截肢。尽管进行了抗生素和手术治疗,病变仍持续出现。败血症筛查为阴性。她随后被诊断为PG,接受类固醇治疗后迅速康复。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2288/5636999/72e30df78fbe/cde-0009-0146-g04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2288/5636999/e0119228f659/cde-0009-0146-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2288/5636999/e65d30fb752a/cde-0009-0146-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2288/5636999/e6f2af23bc59/cde-0009-0146-g03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2288/5636999/72e30df78fbe/cde-0009-0146-g04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2288/5636999/e0119228f659/cde-0009-0146-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2288/5636999/e65d30fb752a/cde-0009-0146-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2288/5636999/e6f2af23bc59/cde-0009-0146-g03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2288/5636999/72e30df78fbe/cde-0009-0146-g04.jpg

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本文引用的文献

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Azathioprine hypersensitivity presenting as cardiogenic shock and Sweet's syndrome in a patient with microscopic polyangiitis.一名显微镜下多血管炎患者出现以心源性休克和Sweet综合征为表现的硫唑嘌呤超敏反应。
Intern Med. 2012;51(14):1889-92. doi: 10.2169/internalmedicine.51.6962. Epub 2012 Jul 15.
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需住院治疗的坏疽性脓皮病:26 例病例报告及随访。
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