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周期性发热与嗜中性皮病:是Sweet综合征吗?

Periodic Fever and Neutrophilic Dermatosis: Is It Sweet's Syndrome?

作者信息

Assari Raheleh, Ziaee Vahid, Parvaneh Nima, Moradinejad Mohammad-Hassan

机构信息

Division of Pediatric Rheumatology, Children's Medical Center, Pediatrics Center of Excellence, Tehran 14194, Iran.

Division of Pediatric Rheumatology, Children's Medical Center, Pediatrics Center of Excellence, Tehran 14194, Iran ; Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran ; Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran.

出版信息

Case Reports Immunol. 2014;2014:320920. doi: 10.1155/2014/320920. Epub 2014 Dec 4.

DOI:10.1155/2014/320920
PMID:25544911
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4273503/
Abstract

A 7-year-old boy with high grade fever (39°C) and warm, erythematous, and indurated plaque above the left knee was referred. According to the previous records of this patient, these indurated plaques had been changed toward abscesses formation and then spontaneous drainage had occurred after about 6 to 7 days, and finally these lesions healed with scars. In multiple previous admissions, high grade fever, leukocytosis, and a noticeable increase in erythrocyte sedimentation rate and C-reactive protein were noted. After that, until 7th year of age, he had shoulder, gluteal, splenic, kidney, and left thigh lesions and pneumonia. The methylprednisolone pulse (30 mg/kg) was initiated with the diagnosis of Sweet's syndrome. After about 10-14 days, almost all of the laboratory data regressed to nearly normal limits. After about 5 months, he was admitted again with tachypnea and high grade fever and leukocytosis. After infusion of one methylprednisolone pulse, the fever and tachypnea resolved rapidly in about 24 hours. In this admission, colchicine (1 mg/kg) was added to the oral prednisolone after discharge. In the periodic fever and neutrophilic dermatosis, the rheumatologist should search for sterile abscesses in other organs.

摘要

一名7岁男孩因高热(39°C)以及左膝上方温暖、红斑样且硬结的斑块前来就诊。根据该患者既往记录,这些硬结斑块会发展为脓肿形成,然后在大约6至7天后自行破溃引流,最终这些病变会留下瘢痕愈合。在之前多次入院时,均发现有高热、白细胞增多,以及红细胞沉降率和C反应蛋白显著升高。此后,直到7岁时,他出现了肩部、臀部、脾脏、肾脏、左大腿病变以及肺炎。诊断为Sweet综合征后开始使用甲泼尼龙冲击治疗(30mg/kg)。大约10 - 14天后,几乎所有实验室数据都恢复到接近正常范围。大约5个月后,他因呼吸急促、高热和白细胞增多再次入院。输注一次甲泼尼龙冲击治疗后,发热和呼吸急促在约24小时内迅速缓解。此次入院时,出院后在口服泼尼松龙基础上加用了秋水仙碱(1mg/kg)。在周期性发热伴嗜中性皮病中,风湿病学家应检查其他器官有无无菌性脓肿。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/616c/4273503/8093e1c8e1cc/CRII2014-320920.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/616c/4273503/d40abbb8736e/CRII2014-320920.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/616c/4273503/8093e1c8e1cc/CRII2014-320920.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/616c/4273503/d40abbb8736e/CRII2014-320920.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/616c/4273503/8093e1c8e1cc/CRII2014-320920.002.jpg

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Autoinflammatory skin disorders in inflammatory bowel diseases, pyoderma gangrenosum and Sweet's syndrome: a comprehensive review and disease classification criteria.炎症性肠病、坏疽性脓皮病和Sweet 综合征中的自身炎症性皮肤病:全面综述和疾病分类标准。
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Autoinflammation and autoimmunity: bridging the divide.
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A case of aseptic abscesses syndrome treated with corticosteroids and TNF-alpha blockade.一例无菌性脓肿综合征经皮质类固醇和 TNF-α 阻断治疗。
Mod Rheumatol. 2013 Jan;23(1):195-9. doi: 10.1007/s10165-012-0640-y. Epub 2012 Apr 20.
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Neonatal Sweet syndrome: a potential marker of serious systemic illness.新生儿Sweet 综合征:严重全身性疾病的潜在标志物。
Pediatrics. 2012 May;129(5):e1353-9. doi: 10.1542/peds.2011-1854. Epub 2012 Apr 16.
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Am J Clin Dermatol. 2012 Jun 1;13(3):191-211. doi: 10.2165/11595240-000000000-00000.
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