背景：荨麻疹性血管炎（UV）是一种罕见的白细胞碎裂性血管炎，其特征是荨麻疹性皮肤病变持续时间长，对治疗的反应差。到目前为止，没有临床指南,诊断标准,或者治疗算法存在，紫外线患者的诊断检查和治疗方法可能在全球范围内有所不同。我们进行了一项在线调查，以检查国际专家如何诊断和治疗紫外线患者，并揭示了管理全球紫外线患者的最大挑战。
方法：问卷的分发包括给世界过敏组织（WAO）数据库中个人的电子邮件，在没有限制的情况下，从属关系，或参与者的国籍（2018年11月）。该电子邮件包含到在线问卷的链接（Internet地址）。回答是匿名的。问卷的链接进一步发送到全球过敏和哮喘欧洲网络（GA2LEN）的荨麻疹参考和卓越中心（UCARE）网络以及土耳其皮肤病学会和日本变态反应学会，他们将链接分发给他们的成员。此外,调查链接发布在俄罗斯变态反应学家和免疫学家协会的在线小组中。
结果：我们从92个国家的医生那里收到了883份完整的调查。据报道，紫外线在临床实践中很少见，平均每名医生每年5名患者。超过三分之二的医生报告了风头，皮肤的燃烧，和残留的色素沉着过度在60-100％的紫外线患者。接受紫外线患者转诊的最常见原因是建立诊断。建立紫外线诊断的最重要特征是持续时间超过24小时的风团（72％），皮肤活检结果（63%），和炎症后色素沉着过度(46%)。紫外线患者最常见的检查是全血细胞计数，红细胞沉降率,C反应蛋白,补体成分,抗核抗体,还有皮肤活检.医生认为紫外线在大多数患者中是未知的原因，和药物和系统性红斑狼疮是最常见的可识别的原因。3名医生中有2名报告说，他们使用标准剂量的第二代抗组胺药作为紫外线患者的一线治疗。在紫外线管理中，最大的挑战是药物的功效有限以及缺乏临床指南和治疗算法。
结论：紫外线是一种具有挑战性的疾病。皮肤活检,紫外线诊断的黄金标准,不是由许多医生执行的。这可能会导致紫外线的误诊，例如,慢性自发性荨麻疹,以及治疗不足。关于紫外线分类以及诊断检查和治疗的国际共识建议，以及评估潜在安全有效治疗紫外线的药物的前瞻性研究，是必要的。
BACKGROUND: Urticarial vasculitis (UV) is a rare type of leukocytoclastic vasculitis characterized by long lasting urticarial skin lesions and poor response to treatment. As of yet, no clinical guidelines, diagnostic criteria, or treatment algorithms exist, and the approaches to the diagnostic workup and treatment of UV patients may differ globally. We conducted an online survey to examine how UV patients are diagnosed and treated by international specialists and to reveal the greatest challenges in managing UV patients worldwide.
METHODS: Distribution of the questionnaire included an email to individuals in the World Allergy Organization (WAO) database, with no restrictions applied to the specialty, affiliation, or nationality of the participants (November 2018). The email contained a link (Internet address) to the online questionnaire. Responses were anonymous. The link to the questionnaire was further sent to the network of Urticaria Centers of Reference and Excellence (UCARE) in the Global Allergy and Asthma European Network (GA2LEN) as well as to the Turkish Dermatology Society and the Japanese Society of Allergology, who distributed the link to their members. In addition, the survey link was posted online in the group of the Russian Society of Allergologists and Immunologists.
RESULTS: We received 883 completed surveys from physicians in 92 countries. UV was reported to be rare in clinical practice, with an average of 5 patients per physician per year. More than two-thirds of physicians reported wheals, burning of the skin, and residual hyperpigmentation in 60-100% of UV patients. The most frequently reported reason for receiving referrals of patients with UV was to establish the diagnosis. The most important features for establishing the diagnosis of UV were wheals of longer than 24 hours duration (72%), the results of skin biopsy (63%), and post-inflammatory hyperpigmentation (46%). The most common tests ordered in UV patients were complete blood count, erythrocyte sedimentation rate, C-reactive protein, complement components, antinuclear antibodies, and skin biopsy. Physicians considered UV to be of unknown cause in most patients, and drugs and systemic lupus erythematosus to be the most common identifiable causes. Two of 3 physicians reported that they use second-generation antihistamines in standard dose as the first-line therapy in patients with UV. The greatest perceived challenges in the management of UV were the limited efficacy of drugs and the absence of clinical guidelines and treatment algorithms.
CONCLUSIONS: UV is a challenging disease. Skin biopsy, a gold standard for UV diagnosis, is not performed by many physicians. This may lead to misdiagnosis of UV, for example, as chronic spontaneous urticaria, and to inadequate treatment. International consensus-based recommendations for the classification of UV and the diagnostic workup and treatment, as well as prospective studies evaluating potentially safe and effective drugs for the treatment of UV, are necessary.
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