{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,22]],"date-time":"2025-11-22T10:36:29Z","timestamp":1763807789506,"version":"build-2065373602"},"reference-count":5,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,5,30]],"date-time":"2025-05-30T00:00:00Z","timestamp":1748563200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["ACR Open Rheumatology"],"published-print":{"date-parts":[[2025,6]]},"abstract":"<jats:p>VEXAS (vacuoles, E1 enzyme, X\u2010linked, autoinflammatory, somatic) syndrome is a rare disorder caused by somatic <jats:italic>UBA1<\/jats:italic> gene mutations, characterized by autoinflammation and hematologic abnormalities, particularly affecting myeloid\u2010lineage progenitors. Sensitive markers include macrocytic anemia, vacuolization of bone marrow precursors, and myelodysplasia. Here, we report the first case of VEXAS syndrome presenting with neutrophilic dermatosis and a serum monoclonal component, without myeloid\u2010lineage hematologic abnormalities atonset. This case underscores the importance of including VEXAS syndrome in the differential diagnosis when monoclonal gammopathy is associated with rheuamtologic features, particularly in older patients presenting with unexplained cutaneous inflammatory manifestations and a serum clonal component, as such presentations may precede the development of classical hematologic abnormalities.<\/jats:p>","DOI":"10.1002\/acr2.70064","type":"journal-article","created":{"date-parts":[[2025,5,31]],"date-time":"2025-05-31T01:39:02Z","timestamp":1748655542000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Unveiling <scp>VEXAS<\/scp> Syndrome: When Skin Manifestations and Monoclonal Gammopathy Precede Myeloid\u2010Lineage Hematologic Abnormality"],"prefix":"10.1002","volume":"7","author":[{"given":"Laura","family":"Di Centa","sequence":"first","affiliation":[{"name":"Division of Rheumatology, Department of Medicine University of Udine, Academic Hospital \u201cSanta Maria della Misericordia,\u201d  Udine Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simone","family":"Longhino","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Medicine University of Udine, Academic Hospital \u201cSanta Maria della Misericordia,\u201d  Udine Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5610-5405","authenticated-orcid":false,"given":"Valeria","family":"Manfr\u00e8","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Medicine University of Udine, Academic Hospital \u201cSanta Maria della Misericordia,\u201d  Udine Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefania","family":"Sacco","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Medicine University of Udine, Academic Hospital \u201cSanta Maria della Misericordia,\u201d  Udine Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luca","family":"Quartuccio","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Medicine University of Udine, Academic Hospital \u201cSanta Maria della Misericordia,\u201d  Udine Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,5,30]]},"reference":[{"issue":"26","key":"e_1_2_4_2_1","first-page":"3591","article-title":"VEXAS syndrome","volume":"137","author":"Grayson PC","year":"2021","journal-title":"Blood"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.27156"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.3390\/biom14060646"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/all.13035"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2665-9913(21)00348-9"}],"container-title":["ACR Open Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr2.70064","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T16:26:07Z","timestamp":1759422367000},"score":1,"resource":{"primary":{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/10.1002\/acr2.70064"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,5,30]]},"references-count":5,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2025,6]]}},"alternative-id":["10.1002\/acr2.70064"],"URL":"https:\/\/doi.org\/10.1002\/acr2.70064","archive":["Portico"],"relation":{},"ISSN":["2578-5745","2578-5745"],"issn-type":[{"type":"print","value":"2578-5745"},{"type":"electronic","value":"2578-5745"}],"subject":[],"published":{"date-parts":[[2025,5,30]]},"assertion":[{"value":"2025-04-07","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-04-09","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-05-30","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70064"}}