{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T20:13:06Z","timestamp":1788207186410,"version":"build-2803163510"},"reference-count":28,"publisher":"Oxford University Press (OUP)","issue":"8","license":[{"start":{"date-parts":[[2024,2,16]],"date-time":"2024-02-16T00:00:00Z","timestamp":1708041600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,7,19]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>VEXAS (Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is caused by acquired somatic mutations in UBA1. Sweet-syndrome-like skin disorders [and especially histiocytoid Sweet syndrome (HSS)] may be associated with VEXAS syndrome.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>To characterize the clinical and histopathological features of HSS in patients with VEXAS syndrome.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>Skin biopsies with a histological diagnosis of HSS at Rennes University Medical Center (Rennes, France) between October 2011 and January 2022 were reviewed in this study. Sanger sequencing and digital polymerase chain reaction were used to screen skin, blood and bone marrow samples for UBA1 variants, and thus classify patients as having VEXAS syndrome or not. We evaluated the clinical, histological and molecular (UBA1) characteristics of patients with or without VEXAS syndrome.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>We compared 15 skin biopsies from 7 patients found to have VEXAS syndrome and 19 skin biopsies from 15 patients without VEXAS syndrome. Persistent C-reactive protein elevation, macrocytosis, anaemia and haematological malignancies were more prevalent in patients with VEXAS syndrome [6\/7 (86%), 6\/7 (86%), 7\/7 (100%) and 6\/7 (86%), respectively] than in patients without [5\/14 (36%), 6\/15 (40%), 8\/15 (53%) and 8\/15 (53%), respectively]. These features sometimes appeared after the first skin manifestations, and a UBA1 mutation was found in the skin of five patients with VEXAS syndrome. Dermal infiltration by reniform histiocytoid cells (myeloperoxidase-positive and\/or CD163-positive) and a periadnexal distribution were more frequently observed in VEXAS syndrome biopsies [15\/15 (100%) and 3\/15 (20%), respectively, vs. 11\/19 (58%) and 0\/19 (0%) in non-VEXAS syndrome biopsies, respectively].<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>Our findings might help pathologists to consider a diagnosis of VEXAS syndrome and to initiate early genetic testing.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ced\/llae015","type":"journal-article","created":{"date-parts":[[2024,2,17]],"date-time":"2024-02-17T07:39:49Z","timestamp":1708155589000},"page":"825-833","source":"Crossref","is-referenced-by-count":10,"title":["Clinical and histological features of histiocytoid Sweet syndrome associated with VEXAS syndrome"],"prefix":"10.1093","volume":"49","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1455-398X","authenticated-orcid":false,"given":"Hortense","family":"Lecoeuvre","sequence":"first","affiliation":[{"name":"CHU Rennes, Dermatology Department , Rennes , France"},{"name":"CHU Rennes, Pathology Department , Rennes , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7ois","family":"Le Gall","sequence":"additional","affiliation":[{"name":"Univ Rennes, CHU Rennes, Pathology Department , Rennes , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9cile","family":"Le Naoures","sequence":"additional","affiliation":[{"name":"CHU Rennes, Pathology Department , Rennes , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie-Dominique","family":"Vignon-Pennamen","sequence":"additional","affiliation":[{"name":"AP-HP, Saint-Louis Hospital, Department of Pathology , Paris , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Lamaison","sequence":"additional","affiliation":[{"name":"Ouest-Pathologie , Rennes , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sol\u00e8ne-Florence","family":"Kammerer-Jacquet","sequence":"additional","affiliation":[{"name":"Univ Rennes, CHU Rennes, Pathology Department , Rennes , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2081-8558","authenticated-orcid":false,"given":"Alain","family":"Lescoat","sequence":"additional","affiliation":[{"name":"Univ Rennes, CHU Rennes, Department of Internal Medicine and Clinical Immunology , INSERM, EHESP, IRSET (Institut de Recherche en Sant\u00e9, Environnement et Travail) - UMR_S 1085, Rennes , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuel","family":"Oger","sequence":"additional","affiliation":[{"name":"Univ Rennes, CHU Rennes, Pharmacovigilance, Pharmacoepidemiology and Drug Information Centre, Department of Clinical Pharmacology , Rennes , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9dric","family":"Pastoret","sequence":"additional","affiliation":[{"name":"CHU Rennes, Hematology Laboratory , Biology Centre, Rennes , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3212-7455","authenticated-orcid":false,"given":"Alain","family":"Dupuy","sequence":"additional","affiliation":[{"name":"Univ Rennes, CHU Rennes, Dermatology Department , Rennes , France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2024,2,16]]},"reference":[{"key":"2024071909342327900_llae015-B1","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1111\/j.1365-2133.1964.tb14541.x","article-title":"An acute febrile neutrophilic 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