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伴 IgG4 相关疾病的低补体血症性皮肤小血管血管炎。

Hypocomplementemic cutaneous small vessel vasculitis in a patient with IgG4-related disease.

机构信息

Department of Dermatology, Fukushima Medical University, Fukushima.

出版信息

Dermatol Online J. 2020 Oct 15;26(10):13030/qt4dg0x6rk.

Abstract

A 62-year-old man was diagnosed with IgG4-related disease based on multiple sites of lymphadenopathy, lymph node biopsy, and elevated serum levels of IgG4. Hypocomplementemia was also observed. During admission, he was referred to our department complaining of multiple areas of purpura on the lower legs. Histological examination revealed leukocytoclastic vasculitis with fibrinoid necrosis, neutrophil infiltration, and nuclear dust in the upper dermis. To date, only a few cases of cutaneous vasculitis have been reported in IgG4-related disease, all of which showed hypocomplementemia. The role of IgG4 in the etiology of leukocytoclastic vasculitis in IgG4-related disease remains unknown and further studies are necessary.

摘要

一位 62 岁男性因多处淋巴结病、淋巴结活检和血清 IgG4 水平升高而被诊断为 IgG4 相关疾病。还观察到低补体血症。住院期间,他因小腿多处紫癜而被转至我科。组织学检查显示真皮上层白细胞碎裂性血管炎伴纤维蛋白样坏死、中性粒细胞浸润和核尘。迄今为止,仅报道了少数 IgG4 相关疾病的皮肤血管炎病例,所有这些病例均显示低补体血症。IgG4 在 IgG4 相关疾病白细胞碎裂性血管炎发病机制中的作用尚不清楚,需要进一步研究。

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