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坏疽性脓皮病作为系统性红斑狼疮的首发临床表现:一例报告及文献综述

Pyoderma gangrenosum, as the first clinical presentation of systemic lupus erythematous, a case report and comprehensive literature review.

作者信息

Rahmani Shahrzad, Goodarzi Azadeh, Akbarzadehpasha Amirhossein, Abri Homa, Sadeghian Amir Mohamad, Behrangi Elham, Laali Azadeh, Shayanfar Nasrin, Lotfi Shiva

机构信息

Department of Rheumatology, Firoozgar Clinical Research Development Center (FCRDC), School of Medicine, Iran University of Medical Sciences, Tehran, Iran.

Department of Dermatology, Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of Medicine, Iran University of Medical Sciences, Tehran, Iran.

出版信息

Caspian J Intern Med. 2025 Jun 23;16(3):577-583. doi: 10.22088/cjim.16.3.577. eCollection 2025 Summer.

DOI:10.22088/cjim.16.3.577
PMID:40786578
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12329354/
Abstract

BACKGROUND

Pyoderma gangrenosum (PG) can be related to a range of systemic and hematologic diseases. The relationship between systemic lupus erythematosus (SLE) and PG has rarely been discussed.

CASE PRESENTATION

In this case study, we report on an SLE patient who presented with PG. The patient is a 53-year-old woman with the chief complaint of developing painful erythematous pustules on her right flank 3 days after hysterectomy surgery. In the span of 3 months, the patient was treated with antibiotics and intra-lesional corticosteroids with no improvement. Meanwhile, she developed symptoms such as polyarthralgia, pleural effusion, hemolytic anemia, and thrombocytopenia, while anti-dsDNA came out positive.

RESULT

Based on pathology results in combination with clinical and para-clinical findings according to the SLICC criteria for SLE 2012, our patient was diagnosed as an active case of SLE presenting with PG. We also searched for and gathered the latest articles on this issue (from 2017 until 2022) to present the most updated review study on the topic in this article.

CONCLUSION

Considering the abundance of PG and SLE concomitance reported cases (more than 30), it might be time to pay more attention to SLE as the underlying cause of PG and keep in mind that any unhealing ulcer in patients with connective tissue disorders should be evaluated for PG. Considering the various treatment options for PG, it is essential that the treatment of choice should cover symptoms of both PG and the underlying disease.

摘要

背景

坏疽性脓皮病(PG)可能与一系列全身性和血液系统疾病相关。系统性红斑狼疮(SLE)与PG之间的关系鲜有讨论。

病例报告

在本病例研究中,我们报告了一名患有PG的SLE患者。该患者为一名53岁女性,主要诉求是子宫切除术后3天右侧胁腹出现疼痛性红斑脓疱。在3个月的时间里,患者接受了抗生素和病灶内注射皮质类固醇治疗,但病情没有改善。与此同时,她出现了多关节痛、胸腔积液、溶血性贫血和血小板减少等症状,同时抗双链DNA呈阳性。

结果

根据2012年SLE的SLICC标准,结合病理结果以及临床和辅助检查结果,我们的患者被诊断为患有PG的活动性SLE病例。我们还搜索并收集了关于这个问题的最新文章(从2017年到2022年),以便在本文中呈现关于该主题的最新综述研究。

结论

鉴于报告的PG与SLE并存病例数量众多(超过30例),或许是时候更多地关注SLE作为PG的潜在病因了,并且要记住,对于结缔组织疾病患者中任何不愈合的溃疡都应评估是否为PG。考虑到PG有多种治疗选择,至关重要的是,所选治疗应涵盖PG和基础疾病的症状。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d8a/12329354/ed62ffc7f074/cjim-16-3-577-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d8a/12329354/1da7354a0fc7/cjim-16-3-577-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d8a/12329354/8c22d4e5f6ad/cjim-16-3-577-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d8a/12329354/a1e0fc092f51/cjim-16-3-577-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d8a/12329354/ed62ffc7f074/cjim-16-3-577-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d8a/12329354/1da7354a0fc7/cjim-16-3-577-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d8a/12329354/8c22d4e5f6ad/cjim-16-3-577-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d8a/12329354/a1e0fc092f51/cjim-16-3-577-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d8a/12329354/ed62ffc7f074/cjim-16-3-577-g004.jpg

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本文引用的文献

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Pyoderma gangrenosum and cobalamin deficiency in systemic lupus erythematosus: a rare but non fortuitous association.系统性红斑狼疮中的坏疽性脓皮病与钴胺素缺乏:一种罕见但并非偶然的关联。
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Ulcerative pyoderma gangrenosum.溃疡性坏疽性脓皮病
CMAJ. 2019 Sep 23;191(38):E1058. doi: 10.1503/cmaj.190721.
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Rev Med Interne. 2020 Jan;41(1):54-57. doi: 10.1016/j.revmed.2019.08.001. Epub 2019 Sep 5.
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Pyoderma gangrenosum masquerading as an ulcer related to antiphospholipid antibodies in a patient with systemic lupus erythematosus.系统性红斑狼疮患者中表现为与抗磷脂抗体相关溃疡的坏疽性脓皮病
Lupus. 2018 Nov;27(13):2174-2176. doi: 10.1177/0961203318793705. Epub 2018 Aug 24.
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Two case reports of pyoderma gangrenosum and systemic lupus erythematosus: A rare but nonfortuitous association?坏疽性脓皮病与系统性红斑狼疮的两例病例报告:一种罕见但并非偶然的关联?
Medicine (Baltimore). 2018 Aug;97(34):e11933. doi: 10.1097/MD.0000000000011933.
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Dysregulation of inflammatory gene expression in lesional and nonlesional skin of patients with pyoderma gangrenosum.坏疽性脓皮病患者皮损及非皮损皮肤中炎症基因表达失调。
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