Chronic recurrent wheals - If not chronic spontaneous urticaria, what else?

Hanna Bonnekoh, Karoline Krause, Pavel Kolkhir
{"title":"Chronic recurrent wheals - If not chronic spontaneous urticaria, what else?","authors":"Hanna Bonnekoh,&nbsp;Karoline Krause,&nbsp;Pavel Kolkhir","doi":"10.5414/ALX02375E","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":7485,"journal":{"name":"Allergologie Select","volume":"7 ","pages":"8-16"},"PeriodicalIF":0.0000,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9835205/pdf/","citationCount":"1","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Allergologie Select","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.5414/ALX02375E","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 1

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.

Abstract Image

慢性复发性荨麻疹-如果不是慢性自发性荨麻疹,还有什么?
慢性荨麻疹,主要是由于慢性自发性荨麻疹(CSU),在一般人群中高达1 - 4%。荨麻疹血管炎(UV)和自身炎症综合征,即冻血素相关周期性综合征(CAPS)和Schnitzler综合征(SchS),可以模拟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任何这些疾病的存在,患者最初表现为慢性荨麻疹。这些症状包括持续时间长(> 24小时)、病灶灼烧、全身性症状和/或炎症标志物(如c反应蛋白、血清淀粉样蛋白A和/或S100A8/9)升高。病变皮肤活检通常表现为白细胞破裂性血管炎(UV)或中性粒细胞浸润(CAPS和SchS)。与CSU相比,第二代H1抗组胺药和omalizumab可以控制大多数患者的症状,但在紫外线和自身炎症性疾病的情况下,分别需要全身免疫抑制和抗白细胞介素(IL)-1治疗。CSU鉴别诊断的稀缺性和低认知度可能与UV、CAPS和SchS患者诊断和治疗的较长延误有关。CSU的鉴别诊断知识很重要,因为只有正确的诊断才能进行适当的治疗。必须考虑和监测并发症,如SchS的淋巴增生性疾病和cap的淀粉样变,以及UV的系统性红斑狼疮等合并症的存在。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 求助全文
来源期刊
自引率
0.00%
发文量
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信