Common variable immunodeficiency (CVID) is a heterogeneous disease characterized by recurrent infections, autoimmunity, malignancies, and granulomatous inflammation. Granulomatous lesion is one of the important manifestations of CVID, which continues to be unknown to many clinicians. While noncaseating granulomatous lesions can be detected in lungs, liver, spleen or conjunctiva of CVID patients, there are only few reported cases with skin granuloma. This report presents a 27-year-old female with multiple persistent cutaneous granulomatous lesions on both hands. The patient had been well until age of 20 years, when she developed these skin lesions and frequent upper respiratory infections and bacterial pneumonia. Also, she experienced recurrent diarrhea (more than 10 episodes). Laboratory evaluation showed decreased serum levels of all immunoglobulin isotypes and low specific antibody responses. The diagnosis of CVID was based on clinical and laboratory findings. Intravenous immunoglobulin therapy at a dosage of 400-500 mg/kg monthly was introduced and improved skin lesions. In conclusion, taking history of recurrent infections and measuring immunoglobulin levels can be suggested in patients with granulomatous lesions instead of other expensive tests.

译文

普通可变免疫缺陷症(CVID)是一种异质性疾病,其特征在于反复感染,自身免疫,恶性肿瘤和肉芽肿性炎症。肉芽肿性病变是CVID的重要表现之一,许多临床医生仍不知道。尽管可以在CVID患者的肺,肝,脾或结膜中检出非干酪性肉芽肿性病变,但报告的皮肤肉芽肿病例很少。该报告介绍了一名27岁的女性,双手多处持续性皮肤肉芽肿性病变。该患者直到20岁时才恢复健康,当时她出现了这些皮肤病变,并频繁出现上呼吸道感染和细菌性肺炎。此外,她经历了反复腹泻(超过10次发作)。实验室评估显示,所有免疫球蛋白同种型的血清水平降低,特异性抗体反应低。 CVID的诊断基于临床和实验室检查结果。引入静脉免疫球蛋白治疗,每月剂量为400-500 mg / kg,可改善皮肤损伤。总之,可以建议对肉芽肿性病变患者进行反复感染史并测量免疫球蛋白水平,而不是其他昂贵的检查方法。

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