Abstract：
Chronic spontaneous urticaria (CSU) and urticarial vasculitis (UV) share several clinical features including the occurrence of wheals. As of yet, the criteria for differentiating the 2 disorders are not clearly defined.
Here, we aimed to identify differences, similarities, and the likelihood for specific clinical features in patients with UV versus those with CSU.
Across 10 Urticaria Centers of Reference and Excellence, 106 patients with skin biopsy-confirmed UV and 126 patients with CSU were prospectively recruited to complete a questionnaire on the clinical features, course, and response to treatment of their disease.
As compared with CSU, patients with UV more often experienced postinflammatory skin hyperpigmentation, wheals of ≥24-hour duration, eye inflammation, and fever (6.9, 4.0, 3.6, and 2.4 times, respectively). Clinical features that increased the risk for UV diagnosis when present at the onset of disease included wheals of ≥24-hour duration (7.3-fold), pain of the skin (7.0-fold), postinflammatory hyperpigmentation (4.1-fold), and fatigue (3.1-fold). The diagnostic delay was markedly longer for normocomplementemic UV as compared with hypocomplementemic UV and CSU (21 vs 5 vs 6 months, respectively). Oral corticosteroids and omalizumab were the most effective treatments in patients with UV and CSU, respectively. Patients with UV showed a higher need for immunosuppressive and anti-inflammatory therapies than patients with CSU.
Long wheal duration, skin pain and hyperpigmentation, and systemic symptoms point to UV rather than CSU as the underlying disease and should prompt further diagnostic workup including a skin biopsy.
Here, we aimed to identify differences, similarities, and the likelihood for specific clinical features in patients with UV versus those with CSU.
Across 10 Urticaria Centers of Reference and Excellence, 106 patients with skin biopsy-confirmed UV and 126 patients with CSU were prospectively recruited to complete a questionnaire on the clinical features, course, and response to treatment of their disease.
As compared with CSU, patients with UV more often experienced postinflammatory skin hyperpigmentation, wheals of ≥24-hour duration, eye inflammation, and fever (6.9, 4.0, 3.6, and 2.4 times, respectively). Clinical features that increased the risk for UV diagnosis when present at the onset of disease included wheals of ≥24-hour duration (7.3-fold), pain of the skin (7.0-fold), postinflammatory hyperpigmentation (4.1-fold), and fatigue (3.1-fold). The diagnostic delay was markedly longer for normocomplementemic UV as compared with hypocomplementemic UV and CSU (21 vs 5 vs 6 months, respectively). Oral corticosteroids and omalizumab were the most effective treatments in patients with UV and CSU, respectively. Patients with UV showed a higher need for immunosuppressive and anti-inflammatory therapies than patients with CSU.
Long wheal duration, skin pain and hyperpigmentation, and systemic symptoms point to UV rather than CSU as the underlying disease and should prompt further diagnostic workup including a skin biopsy.
摘要：
背景：慢性自发性荨麻疹（CSU）和荨麻疹性血管炎（UV）共有几个临床特征，包括风团的发生。到目前为止，区分这两种疾病的标准没有明确定义。
目标：这里，我们的目标是找出差异，相似性,以及紫外线患者与CSU患者的特定临床特征的可能性。
方法：跨越10个荨麻疹参考和卓越中心，前瞻性招募了106例皮肤活检证实为紫外线的患者和126例CSU患者，以完成临床特征问卷，当然,以及对疾病治疗的反应。
结果：与CSU相比，紫外线患者更常经历炎症后皮肤色素沉着过度，≥24小时持续时间的风团，眼部炎症,和发烧（6.9、4.0、3.6和2.4次，分别)。当疾病发作时出现时，增加紫外线诊断风险的临床特征包括≥24小时持续时间的风团（7.3倍），皮肤疼痛（7.0倍），炎症后色素沉着过度（4.1倍），和疲劳（3.1倍）。与低补体紫外线和CSU相比，正常补体紫外线的诊断延迟明显更长（21vs5vs6个月，分别)。口服糖皮质激素和奥马珠单抗是紫外线和CSU患者最有效的治疗方法，分别。与CSU患者相比，UV患者对免疫抑制和抗炎治疗的需求更高。
结论：长风浪持续时间，皮肤疼痛和色素沉着过度，全身性症状表明紫外线而非CSU是潜在疾病，应提示进一步的诊断检查，包括皮肤活检。
目标：这里，我们的目标是找出差异，相似性,以及紫外线患者与CSU患者的特定临床特征的可能性。
方法：跨越10个荨麻疹参考和卓越中心，前瞻性招募了106例皮肤活检证实为紫外线的患者和126例CSU患者，以完成临床特征问卷，当然,以及对疾病治疗的反应。
结果：与CSU相比，紫外线患者更常经历炎症后皮肤色素沉着过度，≥24小时持续时间的风团，眼部炎症,和发烧（6.9、4.0、3.6和2.4次，分别)。当疾病发作时出现时，增加紫外线诊断风险的临床特征包括≥24小时持续时间的风团（7.3倍），皮肤疼痛（7.0倍），炎症后色素沉着过度（4.1倍），和疲劳（3.1倍）。与低补体紫外线和CSU相比，正常补体紫外线的诊断延迟明显更长（21vs5vs6个月，分别)。口服糖皮质激素和奥马珠单抗是紫外线和CSU患者最有效的治疗方法，分别。与CSU患者相比，UV患者对免疫抑制和抗炎治疗的需求更高。
结论：长风浪持续时间，皮肤疼痛和色素沉着过度，全身性症状表明紫外线而非CSU是潜在疾病，应提示进一步的诊断检查，包括皮肤活检。
