PDF(Pubmed)
Abstract：
Chronic urticarial rash, mostly due to chronic spontaneous urticaria (CSU), is seen in up to 1 - 4% of the general population. Urticarial vasculitis (UV) and autoinflammatory syndromes, i.e., cryopyrin-associated periodic syndromes (CAPS) and Schnitzler syndrome (SchS), can mimic CSU-like rash but represent rare disorders with systemic symptoms including fever, headache, conjunctivitis, and arthralgia. Clinical and laboratory features can point to the presence of any of these diseases in patients initially presenting with chronic urticarial rash. These include long-lasting wheals (> 24 hours), lesional burning, systemic symptoms, and/or increase in inflammatory markers (e.g., C-reactive protein, serum amyloid A, and/or S100A8/9). Lesional skin biopsy usually demonstrates leukocytoclastic vasculitis (UV) or neutrophil-rich infiltrate (CAPS and SchS). In contrast to CSU, where second-generation H1 antihistamines and omalizumab allow to control symptoms in most patients, systemic immunosuppression and anti-interleukin (IL)-1 therapies are needed in case of UV and autoinflammatory diseases, respectively. The rarity and low awareness of CSU differential diagnoses may be related to the longer delays in diagnosis and therapy in those affected with UV, CAPS, and SchS. Knowledge of the differential diagnoses of CSU is important because only correct diagnosis allows adequate therapy. Complications such as the development of lymphoproliferative disease in SchS and amyloidosis in CAPS, and the presence of comorbid diseases, such as systemic lupus erythematosus in UV, must be considered and monitored.
摘要：
慢性荨麻疹，主要是由于慢性自发性荨麻疹（CSU），在一般人口的1-4%中可见。荨麻疹性血管炎（UV）和自身炎症综合征，即，冷冻比林相关的周期性综合征（CAPS）和Schnitzler综合征（SchS），可以模仿CSU样皮疹，但代表罕见的全身性症状，包括发烧，头痛,结膜炎,和关节痛.临床和实验室特征可以指出最初出现慢性荨麻疹皮疹的患者中存在任何这些疾病。这些包括持久的风浪（>24小时），损伤性燃烧，全身症状,和/或炎症标志物的增加(例如，C反应蛋白,血清淀粉样蛋白A,和/或S100A8/9)。皮损活检通常显示白细胞碎裂性血管炎（UV）或富含中性粒细胞的浸润（CAPS和SchS）。与CSU相比，第二代H1抗组胺药和奥马珠单抗可以控制大多数患者的症状，在紫外线和自身炎性疾病的情况下，需要全身免疫抑制和抗白细胞介素（IL）-1治疗，分别。CSU鉴别诊断的稀有和低意识可能与受紫外线影响的患者的诊断和治疗延迟更长有关。CAPS,还有SchS.了解CSU的鉴别诊断很重要，因为只有正确的诊断才能进行适当的治疗。并发症，例如SchS中淋巴增生性疾病的发展和CAPS中的淀粉样变性，以及共患疾病的存在，如紫外线下的系统性红斑狼疮，必须考虑和监测。
