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单器官皮肤小血管炎的临床研究

Clinical study on single-organ cutaneous small vessels vasculitis (SoCSVV).

作者信息

Pastuszczak Maciej, Celińska-Löwenhoff Magdalena, Sułowicz Joanna, Wojas-Pelc Anna, Musiał Jacek

机构信息

Department of Dermatology 2nd Department of Medicine, Jagiellonian University Medical College, Cracow, Poland.

出版信息

Medicine (Baltimore). 2017 Mar;96(12):e6376. doi: 10.1097/MD.0000000000006376.

Abstract

Leukocytoclastic vasculitis (LCV) is a heterogenous group of disorders that may manifest as a mild disease isolated to the skin or be a part of life-threatening systemic vasculitis. According to the 2012 Chapel Hill Consensus Conference nomenclature, patients presenting symptoms of LCV confined only to the skin should be defined as suffering from a single-organ cutaneous small vessel vasculitis (SoCSVV). SoCSVV is a benign disease with a good clinical outcome but with a significant risk of relapse and skin ulcer formation.The aim of the current study was to characterize SoCSVV and to identify factors that may be associated with the risk of recurrence and skin ulcers.Medical records of patients with LCV hospitalized at the Department of Dermatology at University Hospital in Cracow in the years 2010 to 2015 were analyzed.A total of 24 patients fulfilled criteria of SoCSVV. Drugs and preceding infections were identified as precipitating factors in 40% and 20% of cases, respectively. Skin lesions other than palpable purpura (i.e., macules, urticarial vasculitis, or ulcers) were identified in almost half of the patients. Interestingly, the presence of macules independently increased the risk of skin ulcer formation (odds ratio = 16; 95% confidence interval: 1.5-176.6; P = 0.0075) in the multivariate logistic regression analysis. One-quarter of patients with SoCSVV experienced relapse during the 6-month follow-up. The greater number of affected skin areas was an independent risk factor of recurrence (odds ratio = 5; 95% confidence interval: 2-45; P = 0.02).SoCSVV was usually associated with drugs and preceding infections. The disease relapses in approximately one-quarter of the patients. The more severe the skin involvement in the course of SoCSVV, the higher is the risk of recurrence.

摘要

白细胞破碎性血管炎(LCV)是一组异质性疾病,可表现为仅累及皮肤的轻症疾病,也可能是危及生命的系统性血管炎的一部分。根据2012年 Chapel Hill 共识会议命名法,仅出现局限于皮肤的LCV症状的患者应被定义为患有单器官皮肤小血管血管炎(SoCSVV)。SoCSVV是一种良性疾病,临床预后良好,但有显著的复发风险和皮肤溃疡形成风险。本研究的目的是对SoCSVV进行特征描述,并确定可能与复发风险和皮肤溃疡相关的因素。对2010年至2015年在克拉科夫大学医院皮肤科住院的LCV患者的病历进行了分析。共有24例患者符合SoCSVV标准。药物和先前感染分别被确定为40%和20%病例的诱发因素。几乎一半的患者出现了除可触及的紫癜以外的皮肤病变(即斑疹、荨麻疹性血管炎或溃疡)。有趣的是,在多因素逻辑回归分析中,斑疹的存在独立增加了皮肤溃疡形成的风险(比值比=16;95%置信区间:1.5 - 176.6;P = 0.0075)。四分之一的SoCSVV患者在6个月的随访期间复发。受影响皮肤区域数量较多是复发的独立危险因素(比值比=5;95%置信区间:2 - 45;P = 0.02)。SoCSVV通常与药物和先前感染有关。大约四分之一的患者疾病复发。SoCSVV病程中皮肤受累越严重,复发风险越高。

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