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低补体血症性荨麻疹性血管炎综合征合并嗜酸性肺炎 1 例:病例报告及文献复习。

A case of hypocomplementemic urticarial vasculitis syndrome complicated by eosinophilic pneumonia: a case report and review of the literature.

机构信息

Department of Dermatology, Dokkyo Medical University School of Medicine, Mibu, Japan.

出版信息

J Int Med Res. 2023 Jul;51(7):3000605231189141. doi: 10.1177/03000605231189141.

Abstract

The primary symptom of urticarial vasculitis (UV), which is a histopathological leukocytoclastic vasculitis disease, is an eruption that resembles urticaria. Other organs may also experience accompanying symptoms. Lung lesions with UV are mostly obstructive pulmonary disease with smoking. However, the coexistence of eosinophilic pneumonia (EP) and complicated UV remains unclear. We report a man in his 70s with chronic obstructive pulmonary disease who attended our department with ring-shaped erythema, marginal edema, and pigmentation. Additionally, a skin histological analysis showed nuclear dust and perivascular neutrophil infiltration, while a blood sample showed a decrease in C3 and C1q concentrations. Administration of prednisone temporarily improved the eruption. However, he developed a cough and a new UV eruption 1 year later. Computed tomography revealed infiltration in the right upper lobe of the lungs, and a blood sample showed a high eosinophil count. He was finally diagnosed with hypocomplementemic urticarial vasculitis syndrome and idiopathic chronic EP. A previous study showed that serum C1q concentrations in patients with EP were lower when this disease was active. Whether a decline in C1q concentrations can cause EP is unclear. However, our case is unique owing to the co-onset of EP with low complement concentrations and recurrence of UV.

摘要

荨麻疹性血管炎(UV)的主要症状是类似于荨麻疹的皮疹,它是一种组织病理学白细胞碎裂性血管炎疾病。其他器官也可能出现伴随症状。与 UV 相关的肺部病变主要是与吸烟有关的阻塞性肺病。然而,嗜酸性粒细胞性肺炎(EP)和复杂的 UV 共存仍不清楚。我们报告了一位 70 多岁的男性,患有慢性阻塞性肺病,他因环状红斑、边缘性水肿和色素沉着到我们科室就诊。此外,皮肤组织学分析显示核尘和血管周围中性粒细胞浸润,而血液样本显示 C3 和 C1q 浓度降低。泼尼松的治疗暂时改善了皮疹。然而,他在 1 年后出现了咳嗽和新的 UV 皮疹。计算机断层扫描显示右肺上叶浸润,血液样本显示嗜酸性粒细胞计数高。他最终被诊断为低补体血症性荨麻疹性血管炎综合征和特发性慢性 EP。先前的一项研究表明,EP 患者在疾病活动期时血清 C1q 浓度较低。C1q 浓度下降是否会导致 EP 尚不清楚。然而,由于 EP 与低补体浓度和 UV 复发同时发生,我们的病例是独特的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a445/10392283/dc2be0759541/10.1177_03000605231189141-fig1.jpg

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