{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T21:02:26Z","timestamp":1793912546714,"version":"4.8.0"},"reference-count":77,"publisher":"MJS Publishing, Medical Journals Sweden AB","license":[{"start":{"date-parts":[[2023,12,19]],"date-time":"2023-12-19T00:00:00Z","timestamp":1702944000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Derm Venereol"],"abstract":"<jats:p>Sweet syndrome is a neutrophilic dermatosis associated with multiple disorders. This retrospective case-series study of patients with Sweet syndrome in a tertiary hospital in Spain from 2001 to 2021, explores clinicopathological characteristics of Sweet syndrome and variables associated with malignancy, presence of autoinflammatory disorders and differences between histological subtypes. A total of 93 patients were identified: 30% idiopathic, 34% malignancy-associated, 29% reactive to infections or drug-associated, and 6% with an autoimmune\/inflammatory condition. Acute myeloid leukaemia was the most common malignancy (16\/93) followed by myelodysplastic syndrome (7\/93). Patients with acute myeloid leukaemia presented isolated flares, marked cytopaenia and rapid response to treatment, whereas myelodysplastic syndrome followed a chronic-recurrent course. The most frequent associated medications and inflammatory\u00a0 disorders were filgrastim and hydroxyurea (n\u2009=\u20092);\u00a0 and inflammatory bowel disease (n\u2009=\u20094). In addition, 3 patients were diagnosed with VEXAS syndrome. Male sex (p\u2009=\u20090.006), fever (p\u2009=\u20090.034), increased erythrocyte sedimentation rate (p\u2009&lt;\u20090.001), anaemia (p\u2009&lt;\u20090.001), and thrombocytopaenia (p\u2009&lt;\u20090.001) were associated with malignancy. Histologically, patients were classified as classic (60%), histiocytoid (22.5%) or subcutaneous (15%), with pain (p\u2009=\u20090.011) and nodules (p\u2009&lt;\u20090.001) being associated with subcutaneous-Sweet syndrome. Sweet syndrome in the context of cytopaenia should alert the presence of malignancy. An\u00a0 acquired autoinflammatory condition should be explored\u00a0 in relapsing Sweet syndrome with myelodysplastic syndrome. A minimum follow-up of 6 months is recommended.<\/jats:p>","DOI":"10.2340\/actadv.v103.18284","type":"journal-article","created":{"date-parts":[[2023,12,19]],"date-time":"2023-12-19T05:55:28Z","timestamp":1702965328000},"page":"adv18284","source":"Crossref","is-referenced-by-count":16,"title":["Sweet Syndrome: Clinical Presentation, Malignancy Association, Autoinflammatory Disorders and Treatment Response in a Cohort of 93 Patients with Long-term Follow-up"],"prefix":"10.2340","volume":"103","author":[{"given":"Javier","family":"Gil-Lianes","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar","family":"Luque-Luna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesc","family":"Alamon-Reig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xavier","family":"Bosch-Amate","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Serra-Garcia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jos\u00e9 M.","family":"Mascar\u00f3 Jr.","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1183","published-online":{"date-parts":[[2023,12,19]]},"reference":[{"key":"44465","unstructured":"Su WP, Liu HN. 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