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皮肤和滑膜出现坏死性及结节病样肉芽肿,与常见变异型免疫缺陷相关。

Necrotizing and sarcoidal granulomas in the skin and synovial membrane, associated with common variable immunodeficiency.

作者信息

Plana Pla A, Bassas-Vila J, Roure S, Ferrándiz C

机构信息

Department of Dermatology, Hospital Universitari Germans Trias i Pujol, Badalona, Spain.

Department of Internal Medicine, Hospital Universitari Germans Trias i Pujol, Badalona, Spain.

出版信息

Clin Exp Dermatol. 2015 Jun;40(4):379-82. doi: 10.1111/ced.12543. Epub 2014 Dec 31.

Abstract

Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinaemia, T-cell abnormalities and recurrent bacterial infections. Patients with CVID can present granulomatous lesions on both the skin and other organs. When these lesions are the first sign of the disease, the diagnosis can be very challenging. We report the case of a patient with undiagnosed CVID, who presented with necrotizing and sarcoidal granulomas on the skin and synovial membrane as the first appearance of immunodeficiency.

摘要

普通可变免疫缺陷(CVID)是一种原发性免疫缺陷,其特征为低丙种球蛋白血症、T细胞异常和反复细菌感染。CVID患者的皮肤和其他器官可出现肉芽肿性病变。当这些病变是该疾病的首发症状时,诊断可能极具挑战性。我们报告了一例未确诊的CVID患者,其皮肤和滑膜出现坏死性和结节病样肉芽肿,这是免疫缺陷的首次表现。

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