Rizwan Ahmad , Jean Dunne , Katie Ridge , Nicole Fagan , Niall Conlon
{"title":"副蛋白阴性il -1介导的炎症性皮肤病:无Gammopathy的schnitzler样综合征的最新进展","authors":"Rizwan Ahmad , Jean Dunne , Katie Ridge , Nicole Fagan , Niall Conlon","doi":"10.1016/j.xjidi.2025.100405","DOIUrl":null,"url":null,"abstract":"<div><div>Schnitzler syndrome (SchS) is a rare autoinflammatory condition characterized by chronic urticaria and systemic inflammation. Obligate diagnostic criteria include the presence of a monoclonal IgM or IgG band, with nearly all cases demonstrating a prompt response to IL-1 blockade. Recently, \"Schnitzler-like\" cases without a paraprotein have been reported. Although the exact nature of their relation to the original eponymous syndrome remains unclear, these cases share similar clinical features and response to IL-1 inhibition. Diagnostic delay is common in autoinflammatory syndromes, and the need to recognize potentially emerging cases is important. We present the case of a male aged 47 years with refractory urticaria, joint pain, and systemic inflammation resembling SchS but without detectable paraprotein, consistent with recently proposed paraprotein-negative IL-1–mediated inflammatory dermatosis (PANID). After failing conventional therapies, the patient achieved rapid and sustained remission with IL-1 blockade. This case underscores the importance of recognizing autoinflammatory syndromes, including PANID, in patients with refractory urticaria with associated inflammatory features. It also highlights the importance of a therapeutic trial of IL-1 inhibition.</div></div>","PeriodicalId":73548,"journal":{"name":"JID innovations : skin science from molecules to population health","volume":"5 6","pages":"Article 100405"},"PeriodicalIF":0.0000,"publicationDate":"2025-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Paraprotein-Negative IL-1–Mediated Inflammatory Dermatosis: An Update on Schnitzler-Like Syndrome in the Absence of a Gammopathy\",\"authors\":\"Rizwan Ahmad , Jean Dunne , Katie Ridge , Nicole Fagan , Niall Conlon\",\"doi\":\"10.1016/j.xjidi.2025.100405\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><div>Schnitzler syndrome (SchS) is a rare autoinflammatory condition characterized by chronic urticaria and systemic inflammation. Obligate diagnostic criteria include the presence of a monoclonal IgM or IgG band, with nearly all cases demonstrating a prompt response to IL-1 blockade. Recently, \\\"Schnitzler-like\\\" cases without a paraprotein have been reported. Although the exact nature of their relation to the original eponymous syndrome remains unclear, these cases share similar clinical features and response to IL-1 inhibition. Diagnostic delay is common in autoinflammatory syndromes, and the need to recognize potentially emerging cases is important. We present the case of a male aged 47 years with refractory urticaria, joint pain, and systemic inflammation resembling SchS but without detectable paraprotein, consistent with recently proposed paraprotein-negative IL-1–mediated inflammatory dermatosis (PANID). After failing conventional therapies, the patient achieved rapid and sustained remission with IL-1 blockade. This case underscores the importance of recognizing autoinflammatory syndromes, including PANID, in patients with refractory urticaria with associated inflammatory features. It also highlights the importance of a therapeutic trial of IL-1 inhibition.</div></div>\",\"PeriodicalId\":73548,\"journal\":{\"name\":\"JID innovations : skin science from molecules to population health\",\"volume\":\"5 6\",\"pages\":\"Article 100405\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-08-20\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"JID innovations : skin science from molecules to population health\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S266702672500061X\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"JID innovations : skin science from molecules to population health","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S266702672500061X","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Paraprotein-Negative IL-1–Mediated Inflammatory Dermatosis: An Update on Schnitzler-Like Syndrome in the Absence of a Gammopathy
Schnitzler syndrome (SchS) is a rare autoinflammatory condition characterized by chronic urticaria and systemic inflammation. Obligate diagnostic criteria include the presence of a monoclonal IgM or IgG band, with nearly all cases demonstrating a prompt response to IL-1 blockade. Recently, "Schnitzler-like" cases without a paraprotein have been reported. Although the exact nature of their relation to the original eponymous syndrome remains unclear, these cases share similar clinical features and response to IL-1 inhibition. Diagnostic delay is common in autoinflammatory syndromes, and the need to recognize potentially emerging cases is important. We present the case of a male aged 47 years with refractory urticaria, joint pain, and systemic inflammation resembling SchS but without detectable paraprotein, consistent with recently proposed paraprotein-negative IL-1–mediated inflammatory dermatosis (PANID). After failing conventional therapies, the patient achieved rapid and sustained remission with IL-1 blockade. This case underscores the importance of recognizing autoinflammatory syndromes, including PANID, in patients with refractory urticaria with associated inflammatory features. It also highlights the importance of a therapeutic trial of IL-1 inhibition.