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Sweet综合征与白塞病的关联:有可能吗?一例病例报告。

Association of Sweet's Syndrome and Behçet's Disease: Is It Possible? A Case Report.

作者信息

Elidrissi El Houcine, Elaissat El Mehdi, Assoufi Naoufal, Tbouda Mohamed

机构信息

Department of Internal Medicine, Military Hospital "Oued DAHAB", Agadir, Morocco.

Anatomo-Pathology Department, Military Hospital "Oued DAHAB", Agadir, Morocco.

出版信息

Eur J Case Rep Intern Med. 2023 Sep 19;10(10):004073. doi: 10.12890/2023_004073. eCollection 2023.

DOI:10.12890/2023_004073
PMID:37789984
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10545150/
Abstract

INTRODUCTION

Behçet's disease is a systemic vasculitis characterized by a large clinical polymorphism with a particular frequency of cutaneous signs. Sweet's syndrome is a neutrophilic dermatosis marked by the sudden appearance of painful skin lesions in the form of erythematous papules, nodules or plaques. This syndrome is associated with high fever, neutrophilia and histologically a diffuse infiltrate of neutrophils in the dermis.

OBSERVATION

We report the case of a 43-year-old patient followed for Behçet's disease, who developed cutaneous plaques of neutrophilic dermatosis of both upper limbs. The clinical and biological picture was in favor of Sweet's syndrome.

CONCLUSION

The coexistence of Sweet's syndrome and Behçet's disease is already reported in the literature. The association is however very rare given the differences in the clinical and pathogenic features between the two conditions.

LEARNING POINTS

The appearance of neutrophilic dermatosis during a skin flare-up of Behçet's disease alerted us to a possible link between Sweet's syndrome and Behçet's disease.The morphology of the skin lesions associated with these pathologies is heterogeneous, making diagnosis sometimes difficult.Cases reported in the literature concerning the association between Sweet's syndrome and Behçet's disease are rare.

摘要

引言

白塞病是一种全身性血管炎,其临床症状具有高度多样性,皮肤症状出现频率较高。Sweet综合征是一种嗜中性皮病,其特征为突然出现以红斑丘疹、结节或斑块形式存在的疼痛性皮肤损害。该综合征伴有高热、嗜中性粒细胞增多,组织学表现为真皮内嗜中性粒细胞弥漫浸润。

病例报告

我们报告一例43岁白塞病患者,其双上肢出现嗜中性皮病性皮肤斑块。临床和生物学表现支持Sweet综合征。

结论

文献中已报道过Sweet综合征与白塞病共存的情况。鉴于这两种疾病在临床和致病特征上存在差异,这种关联非常罕见。

经验教训

白塞病皮肤发作时出现嗜中性皮病提醒我们Sweet综合征与白塞病之间可能存在联系。与这些病症相关的皮肤损害形态多样,有时诊断困难。文献中报道的Sweet综合征与白塞病关联的病例很少。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cbf3/10545150/e1ef82a995f4/4073_Fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cbf3/10545150/2163778ebb86/4073_Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cbf3/10545150/0ea3617af0ad/4073_Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cbf3/10545150/e1ef82a995f4/4073_Fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cbf3/10545150/2163778ebb86/4073_Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cbf3/10545150/0ea3617af0ad/4073_Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cbf3/10545150/e1ef82a995f4/4073_Fig3.jpg

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Sweet's syndrome in Behçet's disease.白塞病中的斯威特综合征。
Joint Bone Spine. 2007 Oct;74(5):495-6. doi: 10.1016/j.jbspin.2006.11.022. Epub 2007 Jul 9.
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Sweet's syndrome--a comprehensive review of an acute febrile neutrophilic dermatosis.斯威特综合征——急性发热性嗜中性皮病的全面综述
Orphanet J Rare Dis. 2007 Jul 26;2:34. doi: 10.1186/1750-1172-2-34.
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Expression of cytokines, chemokines, and chemokine receptors in oral ulcers of patients with Behcet's disease (BD) and recurrent aphthous stomatitis is Th1-associated, although Th2-association is also observed in patients with BD.白塞病(BD)和复发性阿弗他口炎患者口腔溃疡中细胞因子、趋化因子及趋化因子受体的表达与Th1相关,不过在BD患者中也观察到了与Th2的关联。
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