{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T18:47:38Z","timestamp":1784918858810,"version":"3.55.0"},"reference-count":26,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2022,1,20]],"date-time":"2022-01-20T00:00:00Z","timestamp":1642636800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>S\u00e9zary syndrome is a rare subtype of cutaneous T-cell lymphoma characterized by erythroderma, peripheral lymphadenopathies, and circulating atypical cerebriform T-cells. To date, no definite staging system has been developed for these patients. In this retrospective analysis of the archive of the Dermatological Clinic of the University of Turin, Italy, erythrodermic SS patients were classified according to clinical records and photographs into three main presentations: erythematous, infiltrated, or melanodermic. The pattern of erythroderma was found to be associated with disease outcome, as better survivals were recorded in patients with erythematous and infiltrative erythroderma. Patients in the melanodermic group, though less represented in our investigation, seemed to show a worse trend in survival. According to this preliminary evidence, a new prognostic classification, with a revised score specific for S\u00e9zary syndrome patients, can be proposed to usefully integrate the current staging system. The correlation displayed in our research will be hopefully confirmed by prospective studies with larger cohorts, with the aim of identifying significant prognostic features in this subset of cutaneous T-cell lymphoma patients.<\/jats:p>","DOI":"10.3390\/cells11030333","type":"journal-article","created":{"date-parts":[[2022,1,20]],"date-time":"2022-01-20T22:40:20Z","timestamp":1642718420000},"page":"333","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["S\u00e9zary Syndrome: Different Erythroderma Morphological Features with Proposal for a Clinical Score System"],"prefix":"10.3390","volume":"11","author":[{"given":"Gabriele","family":"Roccuzzo","sequence":"first","affiliation":[{"name":"Section of Dermatology, Department of Medical Sciences, University of Turin, 10126 Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Giordano","sequence":"additional","affiliation":[{"name":"Section of Dermatology, Department of Medical Sciences, University of Turin, 10126 Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7253-2370","authenticated-orcid":false,"given":"Gianluca","family":"Avallone","sequence":"additional","affiliation":[{"name":"Section of Dermatology, Department of Medical Sciences, University of Turin, 10126 Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1706-656X","authenticated-orcid":false,"given":"Marco","family":"Rubatto","sequence":"additional","affiliation":[{"name":"Section of Dermatology, Department of Medical Sciences, University of Turin, 10126 Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Canonico","sequence":"additional","affiliation":[{"name":"Section of Dermatology, Department of Medical Sciences, University of Turin, 10126 Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6341-1817","authenticated-orcid":false,"given":"Ada","family":"Funaro","sequence":"additional","affiliation":[{"name":"Laboratory of Immunogenetics, Department of Medical Sciences, University of Turin, 10126 Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2287-9020","authenticated-orcid":false,"given":"Erika","family":"Ortolan","sequence":"additional","affiliation":[{"name":"Laboratory of Immunogenetics, Department of Medical Sciences, University of Turin, 10126 Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebecca","family":"Senetta","sequence":"additional","affiliation":[{"name":"Pathology Unit, Department of Oncology, University of Turin, 10126 Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Fava","sequence":"additional","affiliation":[{"name":"Section of Dermatology, Department of Medical Sciences, University of Turin, 10126 Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Teresa","family":"Fierro","sequence":"additional","affiliation":[{"name":"Section of Dermatology, Department of Medical Sciences, University of Turin, 10126 Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simone","family":"Ribero","sequence":"additional","affiliation":[{"name":"Section of Dermatology, Department of Medical Sciences, University of Turin, 10126 Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pietro","family":"Quaglino","sequence":"additional","affiliation":[{"name":"Section of Dermatology, Department of Medical Sciences, University of Turin, 10126 Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,1,20]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"3768","DOI":"10.1182\/blood-2004-09-3502","article-title":"WHO-EORTC classification for cutaneous lymphomas","volume":"105","author":"Willemze","year":"2005","journal-title":"Blood"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"4730","DOI":"10.1200\/JCO.2009.27.7665","article-title":"Survival outcomes and prognostic factors in mycosis fungoides\/Sezary syndrome: Validation of the revised International Society of Cutaneous Lymphomas\/European Organisation for Research and Treatment of cancer staging proposal","volume":"28","author":"Agar","year":"2010","journal-title":"J. Clin. Oncol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1713","DOI":"10.1182\/blood-2007-03-055749","article-title":"Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: A proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC)","volume":"110","author":"Olsen","year":"2007","journal-title":"Blood"},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Olsen, E.A., Whittaker, S., Willemze, R., Pinter-Brown, L., Foss, F.M., Geskin, L.J., Schwartz, L.H., Horwitz, S.M., Guitart, J., and Zic, J. (2021). Primary Cutaneous Lymphoma: Recommendations for Clinical Trial Design and Staging Update from the ISCL, USCLC, and EORTC. Blood.","DOI":"10.1182\/blood.2021012057"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1016\/j.jaad.2014.11.015","article-title":"S\u00e9zary syndrome without erythroderma","volume":"72","author":"Henn","year":"2015","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1016\/j.jaad.2017.05.036","article-title":"Early clinical manifestations of S\u00e9zary syndrome: A multicenter retrospective cohort study","volume":"77","author":"Mangold","year":"2017","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1309\/AJCP1NA3YCHCDEIG","article-title":"Blood flow cytometry in S\u00e9zary syndrome: New insights on prognostic relevance and immunophenotypic changes during follow-up","volume":"143","author":"Novelli","year":"2015","journal-title":"Am. J. Clin. Pathol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/j.ejca.2018.01.076","article-title":"Blood classification and blood response criteria in mycosis fungoides and S\u00e9zary syndrome using flow cytometry: Recommendations from the EORTC cutaneous lymphoma task force","volume":"93","author":"Scarisbrick","year":"2018","journal-title":"Eur. J. Cancer"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1046\/j.1365-2133.2001.04014.x","article-title":"The relevance of the CD4+ CD26\u2014subset in the identification of circulating S\u00e9zary cells","volume":"144","author":"Bernengo","year":"2001","journal-title":"Br. J. Dermatol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1364","DOI":"10.1016\/j.jid.2016.01.038","article-title":"Evaluation of Immunophenotypic and Molecular Biomarkers for S\u00e9zary Syndrome Using Standard Operating Procedures: A Multicenter Study of 59 Patients","volume":"136","author":"Boonk","year":"2016","journal-title":"J. Investig. Dermatol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"562","DOI":"10.1080\/10428194.2017.1347650","article-title":"Current Systemic Therapeutic Options for Advanced Mycosis Fungoides and S\u00e9zary Syndrome","volume":"59","author":"Janiga","year":"2018","journal-title":"Leuk. Lymphoma"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/jdv.12311","article-title":"Guidelines on the use of extracorporeal photopheresis","volume":"28","author":"Knobler","year":"2014","journal-title":"J. Eur. Acad Dermatol. Venereol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.ejca.2017.02.027","article-title":"European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides\/S\u00e9zary syndrome\u2014Update 2017","volume":"77","author":"Trautinger","year":"2017","journal-title":"Eur. J. Cancer"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1016\/j.jid.2020.07.026","article-title":"Phenotypical Markers, Molecular Mutations, and Immune Microenvironment as Targets for New Treatments in Patients with Mycosis Fungoides and\/or S\u00e9zary Syndrome","volume":"141","author":"Quaglino","year":"2021","journal-title":"J. Investig. Dermatol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1038\/s41572-021-00296-9","article-title":"Cutaneous T cell lymphoma","volume":"7","author":"Dummer","year":"2021","journal-title":"Nat. Rev. Dis. Prim."},{"key":"ref_16","doi-asserted-by":"crossref","unstructured":"Dumont, M., de Latour, R.P., Ram-Wolff, C., Bagot, M., and de Masson, A. (2020). Allogeneic Hematopoietic Stem Cell Transplantation in Cutaneous T-Cell Lymphomas. Cancers, 12.","DOI":"10.3390\/cancers12102856"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"522","DOI":"10.6004\/jnccn.2020.0022","article-title":"NCCN Guidelines Insights: Primary Cutaneous Lymphomas, Version 2.2020","volume":"18","author":"Horwitz","year":"2020","journal-title":"J. Natl. Compr. Cancer Netw."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1111\/bjd.17523","article-title":"Updates in Cutaneous Lymphoma: Evidence-Based Guidelines for the Management of Cutaneous Lymphoma 2018","volume":"180","author":"Wang","year":"2019","journal-title":"Br. J. Dermatol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"733770","DOI":"10.3389\/fonc.2021.733770","article-title":"Immune Check Point Inhibitors in Primary Cutaneous T-Cell Lymphomas: Biologic Rationale, Clinical Results and Future Perspectives","volume":"11","author":"Roccuzzo","year":"2021","journal-title":"Front. Oncol."},{"key":"ref_20","first-page":"492","article-title":"H\u00e4mosiderimpigmentierung beim S\u00e9zary-Syndrom [Hemosiderin pigmentation in Sez\u00e1ry's syndrome]","volume":"27","author":"Hundeiker","year":"1976","journal-title":"Hautarzt"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1217","DOI":"10.1016\/S0190-9622(86)70294-6","article-title":"Histopathologic studies in Sezary syndrome and erythrodermic mycosis fungoides: A comparison with benign forms of erythroderma","volume":"15","author":"Sentis","year":"1986","journal-title":"J. Am. Acad Dermatol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1111\/j.1600-0560.1997.tb00793.x","article-title":"Cutaneous histopathology of S\u00e9zary syndrome: A study of 41 cases with a proven circulating T-cell clone","volume":"24","author":"Trotter","year":"1997","journal-title":"J. Cutan. Pathol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1111\/bjd.13832","article-title":"Histopathological and immunophenotypical criteria for the diagnosis of S\u00e9zary syndrome in differentiation from other erythrodermic skin diseases: A European Organisation for Research and Treatment of Cancer (EORTC) Cutaneous Lymphoma Task Force Study of 97 cases","volume":"173","author":"Klemke","year":"2015","journal-title":"Br. J. Dermatol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1002\/hon.2832","article-title":"Critical concepts and management recommendations for cutaneous T-cell lymphoma: A consensus-based position paper from the Italian Group of Cutaneous Lymphoma","volume":"39","author":"Zinzani","year":"2021","journal-title":"Hematol. Oncol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"2598","DOI":"10.1200\/JCO.2010.32.0630","article-title":"Clinical end points and response criteria in mycosis fungoides and S\u00e9zary syndrome: A consensus statement of the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer","volume":"29","author":"Olsen","year":"2011","journal-title":"J. Clin. Oncol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1016\/j.jaad.2011.06.014","article-title":"Peripheral blood findings in erythrodermic patients: Importance for the differential diagnosis of S\u00e9zary syndrome","volume":"66","author":"Nagler","year":"2012","journal-title":"J. Am. Acad. Dermatol."}],"container-title":["Cells"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4409\/11\/3\/333\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T22:04:20Z","timestamp":1760133860000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4409\/11\/3\/333"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1,20]]},"references-count":26,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2022,2]]}},"alternative-id":["cells11030333"],"URL":"https:\/\/doi.org\/10.3390\/cells11030333","relation":{},"ISSN":["2073-4409"],"issn-type":[{"value":"2073-4409","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,1,20]]}}}