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Abstract：
BACKGROUND: Urticarial vasculitis (UV) should be differentiated from chronic spontaneous urticaria (CSU) in patients initially presenting with recurrent wheals, although criteria for differential diagnosis remain ill-defined.
OBJECTIVE: To set the goals, define criteria and unmet needs in UV diagnosis and differential diagnosis with CSU, and explore the possibility of coexistence of both diseases.
METHODS: Thirteen experts experienced in UV research participated in a Delphi survey of European Academy of Allergy and Clinical Immunology taskforce. This Delphi survey involved three rounds of anonymous responses to n = 32 questions with the aim to aggregate the experts\' opinions and to achieve consensus. Urticaria specialists (n = 130, most from Urticaria Centers of Reference and Excellence) evaluated the consensus statements and recommendations in the fourth and final round.
RESULTS: The panel agreed that essential criteria to guide a skin biopsy in patients with recurrent wheals should include at least one of the following features: wheal duration >24 h, bruising/postinflammatory hyperpigmentation, and systemic symptoms. Leukocytoclasia and fibrin deposits were identified as a minimum set of UV histological criteria. As agreed by the panel members, CSU and normocomplementemic UV (NUV) may coexist in some patients.
CONCLUSIONS: The use of established criteria for the diagnosis and differential diagnosis of UV in patients with recurrent wheals can help guide the diagnostic approach and prompt earlier treatment. Further studies should investigate whether CSU and NUV are different entities or part of a disease spectrum.
OBJECTIVE: To set the goals, define criteria and unmet needs in UV diagnosis and differential diagnosis with CSU, and explore the possibility of coexistence of both diseases.
METHODS: Thirteen experts experienced in UV research participated in a Delphi survey of European Academy of Allergy and Clinical Immunology taskforce. This Delphi survey involved three rounds of anonymous responses to n = 32 questions with the aim to aggregate the experts\' opinions and to achieve consensus. Urticaria specialists (n = 130, most from Urticaria Centers of Reference and Excellence) evaluated the consensus statements and recommendations in the fourth and final round.
RESULTS: The panel agreed that essential criteria to guide a skin biopsy in patients with recurrent wheals should include at least one of the following features: wheal duration >24 h, bruising/postinflammatory hyperpigmentation, and systemic symptoms. Leukocytoclasia and fibrin deposits were identified as a minimum set of UV histological criteria. As agreed by the panel members, CSU and normocomplementemic UV (NUV) may coexist in some patients.
CONCLUSIONS: The use of established criteria for the diagnosis and differential diagnosis of UV in patients with recurrent wheals can help guide the diagnostic approach and prompt earlier treatment. Further studies should investigate whether CSU and NUV are different entities or part of a disease spectrum.
摘要：
背景：对于最初出现复发性风团的患者，应将荨麻疹性血管炎（UV）与慢性自发性荨麻疹（CSU）区分开来，尽管鉴别诊断的标准仍不明确。
目标：为了设定目标，用CSU定义紫外线诊断和鉴别诊断的标准和未满足的需求，探索两种疾病共存的可能性。
方法：13位具有紫外线研究经验的专家参加了欧洲过敏和临床免疫学学会工作组的德尔菲调查。这项德尔福调查涉及对n=32个问题的三轮匿名回答，目的是汇总专家的意见并达成共识。荨麻疹专家（n=130，大多数来自荨麻疹参考和卓越中心）在第四轮也是最后一轮评估了共识声明和建议。
结果：专家组一致认为，指导复发风团患者皮肤活检的基本标准应至少包括以下特征之一：风团持续时间>24小时，瘀伤/炎症后色素沉着过度，和全身症状。白细胞扩张和纤维蛋白沉积被鉴定为UV组织学标准的最小集合。根据小组成员的同意，CSU和正常补体紫外线（NUV）在某些患者中可能共存。
结论：使用已建立的标准对复发性风团患者的紫外线诊断和鉴别诊断可以帮助指导诊断方法并促进早期治疗。进一步的研究应该调查CSU和NUV是不同的实体还是疾病谱的一部分。
目标：为了设定目标，用CSU定义紫外线诊断和鉴别诊断的标准和未满足的需求，探索两种疾病共存的可能性。
方法：13位具有紫外线研究经验的专家参加了欧洲过敏和临床免疫学学会工作组的德尔菲调查。这项德尔福调查涉及对n=32个问题的三轮匿名回答，目的是汇总专家的意见并达成共识。荨麻疹专家（n=130，大多数来自荨麻疹参考和卓越中心）在第四轮也是最后一轮评估了共识声明和建议。
结果：专家组一致认为，指导复发风团患者皮肤活检的基本标准应至少包括以下特征之一：风团持续时间>24小时，瘀伤/炎症后色素沉着过度，和全身症状。白细胞扩张和纤维蛋白沉积被鉴定为UV组织学标准的最小集合。根据小组成员的同意，CSU和正常补体紫外线（NUV）在某些患者中可能共存。
结论：使用已建立的标准对复发性风团患者的紫外线诊断和鉴别诊断可以帮助指导诊断方法并促进早期治疗。进一步的研究应该调查CSU和NUV是不同的实体还是疾病谱的一部分。
