{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T20:26:45Z","timestamp":1791404805082,"version":"4.3.3"},"reference-count":35,"publisher":"American Medical Association (AMA)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAMA Dermatol"],"published-print":{"date-parts":[[2026,1,1]]},"abstract":"\n Importance<\/jats:title>\n Immune-mediated inflammatory diseases are rare but increasingly reported among patients with myelodysplastic syndromes (MDS) or chronic myelomonocytic leukemia (CMML). Systemic lupus erythematosus (LE) and cutaneous LE associated with MDS\/CMML have been rarely described, with atypical features and refractory disease.<\/jats:p>\n <\/jats:sec>\n \n Objective<\/jats:title>\n To provide a comprehensive description of the phenotype and therapeutic responses of LE associated with MDS\/CMML and to compare them with idiopathic LE.<\/jats:p>\n <\/jats:sec>\n \n Design, Setting, and Participants<\/jats:title>\n This retrospective case-control study included nationwide, multicenter data from January 1975 to January 2023. Patients with MDS\/CMML who either fulfilled classification criteria for systemic LE or had skin lesions diagnosed as cutaneous LE were included. For MDS\/CMML systemic LE, a 2:1 case-control study was conducted with idiopathic systemic LE. Clinical features, centralized skin histopathology, and targeted next-generation sequencing were analyzed. Data were analyzed from May 2022 to June 2025.<\/jats:p>\n <\/jats:sec>\n \n Main Outcomes and Measures<\/jats:title>\n The clinical, pathological, and molecular features of LE occurring in the setting of MDS or CMML compared with idiopathic LE.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Of 24 included patients, 9 (38%) were female, 15 (63%) were male, and the median (range) age at diagnosis was 65 (32-85) years. A total of 19 were diagnosed with systemic LE and 5 with cutaneous LE. The median (range) follow-up was 4.5 (1-31) years. Cutaneous involvement was the most common manifestation of LE (17 [71%]). Chilblain lupus was the predominant subtype (6 [35%]). Compared with idiopathic systemic LE, patients with MDS\/CMML\u2013associated LE were older (median [range] age, 65 [32-85] years vs 23 [11-55] years;\n P<\/jats:italic>\n \u2009&amp;lt;\u2009.001), more frequently male (10 [53%] vs 3 [8%];\n P<\/jats:italic>\n \u2009=\u2009.008), had less kidney involvement (2 [10%] vs 27 [71%];\n P<\/jats:italic>\n \u2009&amp;lt;\u2009.001), had less articular involvement (7 [36%] vs 37 [97%];\n P<\/jats:italic>\n \u2009&amp;lt;\u2009.001), and had reduced anti\u2013double-stranded DNA positivity (6 [32%] vs 29 [76%];\n P<\/jats:italic>\n \u2009=\u2009.001). The underlying hematologic diseases included MDS (16 [66%]) and CMML (8 [34%]), with 22 (92%) classified as lower risk (Revised International Prognostic Scoring System score of 3.5 or less). Centralized histopathological review reclassified 6 skin biopsies (50%) as MDS\/CMML cutis. Identical myeloid variants were detected in blood and skin in 6 of 8 patients, supporting a clonal inflammatory process. Standard LE therapies were often poorly effective, while clone-directed therapies (azacitidine or allogeneic hematopoietic stem cell transplant) led to parallel hematologic and LE responses in 5 of 7 patients.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions and Relevance<\/jats:title>\n In this study, MDS\/CMML\u2013associated lupuslike manifestations were a distinct entity mimicking systemic LE or cutaneous LE and characterized by clonal inflammation rather than classic autoimmunity in most cases. Early recognition is important, as treatment may require clone-targeting therapies rather than conventional LE therapy.<\/jats:p>\n <\/jats:sec>","DOI":"10.1001\/jamadermatol.2025.4586","type":"journal-article","created":{"date-parts":[[2025,11,19]],"date-time":"2025-11-19T16:00:33Z","timestamp":1763568033000},"page":"31","source":"Crossref","is-referenced-by-count":1,"title":["Lupuslike Manifestations in Myelodysplastic Syndromes and Chronic Myelomonocytic Leukemia"],"prefix":"10.1001","volume":"162","author":[{"given":"Jeanne","family":"Chauffier","sequence":"first","affiliation":[{"name":"Internal Medicine Department, H\u00f4pital Saint-Antoine, Assistance Publique-H\u00f4pitaux de Paris, Sorbonne University, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Jachiet","sequence":"additional","affiliation":[{"name":"Internal Medicine Department, H\u00f4pital Saint-Antoine, Assistance Publique-H\u00f4pitaux de Paris, Sorbonne University, 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