{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T18:09:11Z","timestamp":1787162951551,"version":"build-2736575974"},"reference-count":92,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2026,1,16]],"date-time":"2026-01-16T00:00:00Z","timestamp":1768521600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dermatopathology"],"abstract":"<jats:p>Background: Beh\u00e7et-like syndrome (BLS) refers to the presence of Beh\u00e7et\u2019s disease (BD) features occurring in association with distinct clinical\u2013pathological conditions such as inborn errors of immunity, myeloproliferative disorders, infections, or drug exposure. BLS may differ clinically from BD and is increasingly recognized as a separate entity. Distinguishing BLS from primary BD is essential for appropriate management, and studying BLS may provide insights into BD pathogenesis. Objectives: To summarize clinical features, treatments, and genetic abnormalities reported in BLS, we reviewed all published cases up to January 2024. Methods: A systematic search of PubMed, Scopus, and Embase was performed using the terms \u201cBeh\u00e7et-like syndrome\u201d, \u201cBeh\u00e7et-like disease\u201d, and \u201cPseudo-Beh\u00e7et disease\u201d. We included English-language reports of patients &gt; 12 years old with a defined underlying etiology and Beh\u00e7et-like manifestations, defined by \u22652 ICBD criteria and\/or gastrointestinal involvement, mucosal ulcers, thrombosis, or non-recurrent disease. Epidemiological, clinical, laboratory, histological, and treatment data were extracted and analyzed descriptively. Results: Of 679 publications, 53 met inclusion criteria, comprising 100 patients with BLS. The median age was 44 years (IQR 22\u201352), with a female predominance (1:2). Fifty-three percent were from non-European countries. A genetic disorder was identified in 70% of cases, while HLA-B51 was present in 10%. Frequent manifestations included skin lesions (68%), fever (56%), intestinal involvement (43%), and joint symptoms (43%). Treatments included glucocorticoids (65%), conventional DMARDs (32%), and biologics (22%), mainly anti-TNF agents. Antiviral\/antibiotic therapy was used in 9% and chemotherapy in 15%. Two patients with trisomy-8 MDS underwent allogeneic stem cell transplantation. Conclusions: Diverse conditions\u2014including monogenic diseases, immune defects, myeloproliferative disorders, infections, and drug-related reactions\u2014can produce Beh\u00e7et-like features. Our findings highlight differences in clinical expression and treatment response across BLS etiologies. Recognizing BLS is essential for appropriate management and may contribute to a deeper understanding of BD pathogenesis and future targeted therapies.<\/jats:p>","DOI":"10.3390\/dermatopathology13010007","type":"journal-article","created":{"date-parts":[[2026,1,16]],"date-time":"2026-01-16T14:46:33Z","timestamp":1768574793000},"page":"7","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Beh\u00e7et-like Syndromes: A Comprehensive Review"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8691-6826","authenticated-orcid":false,"given":"Gaia","family":"Mancuso","sequence":"first","affiliation":[{"name":"Allergy and Clinical Immunology Service, Regional Hospital of Locarno, Ente Ospedaliero Cantonale (EOC), 6600 Locarno, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Igor","family":"Salvad\u00e8","sequence":"additional","affiliation":[{"name":"Allergy and Clinical Immunology Service, Regional Hospital of Locarno, Ente Ospedaliero Cantonale (EOC), 6600 Locarno, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1089-2511","authenticated-orcid":false,"given":"Adam","family":"Ogna","sequence":"additional","affiliation":[{"name":"Department of General Internal Medicine, Regional Hospital of Locarno, Ente Ospedaliero Cantonale (EOC), 6600 Locarno, Switzerland"},{"name":"Faculty of Biomedical Sciences, Universit\u00e0 della Svizzera Italiana (USI), 6962 Lugano, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brenno","family":"Balestra","sequence":"additional","affiliation":[{"name":"Department of General Internal Medicine, Regional Hospital of Mendrisio, Ente Ospedaliero Cantonale (EOC), 6850 Mendrisio, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8179-1793","authenticated-orcid":false,"given":"Helmut","family":"Beltraminelli","sequence":"additional","affiliation":[{"name":"Faculty of Biomedical Sciences, Universit\u00e0 della Svizzera Italiana (USI), 6962 Lugano, Switzerland"},{"name":"Dermatology and Dermatopathology Unit, Ente Ospedaliero Cantonale (EOC), 6500 Bellinzona, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,1,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e12973","DOI":"10.1111\/sji.12973","article-title":"Beh\u00e7et disease (BD) and BD-like clinical phenotypes: NF-\u03baB pathway in mucosal ulcerating diseases","volume":"92","author":"Perazzio","year":"2020","journal-title":"Scand. 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