Sweet syndrome: long-term follow-up of 138 patients

Summary Background Several studies support a strong association of Sweet syndrome (SS) with malignancy. However, only a few studies analysing the clinical features of malignancy‐associated SS have been published in recent years. Aim To retrospectively study the clinical features of SS that could pre...

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Veröffentlicht in:Clinical and experimental dermatology 2016-10, Vol.41 (7), p.741-746
Hauptverfasser: Marcoval, J., Martín-Callizo, C., Valentí-Medina, F., Bonfill-Ortí, M., Martínez-Molina, L.
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container_end_page 746
container_issue 7
container_start_page 741
container_title Clinical and experimental dermatology
container_volume 41
creator Marcoval, J.
Martín-Callizo, C.
Valentí-Medina, F.
Bonfill-Ortí, M.
Martínez-Molina, L.
description Summary Background Several studies support a strong association of Sweet syndrome (SS) with malignancy. However, only a few studies analysing the clinical features of malignancy‐associated SS have been published in recent years. Aim To retrospectively study the clinical features of SS that could predict the development of associated malignancies and to analyse the development of malignant neoplasia during long‐term follow‐up of patients with SS. Methods Clinical features of the patients diagnosed with SS syndrome between 1987 and 2013 at Bellvitge Hospital (Barcelona, Spain) were retrospectively analysed. Results In total, 138 patients were included in the study (66 male, 72 female, mean ± SD age 51.24 ± 14.11 years). SS was associated with haematological malignancy in 31 cases, infection in 23, inflammatory bowel disease in 12, inflammatory systemic disease in 8, and solid tumours in 4. It was drug‐induced in 5 cases and idiopathic in 54. In four patients, an underlying haematological disease that was considered related to SS was diagnosed between 4 and 16 months after SS presentation. Variables significantly associated with malignancy in multivariate logistic regression analysis were age (OR = 1.08 for each increasing year, P = 0.01), anaemia (OR = 9.38, P = 0.001), thrombocytopenia (OR = 16.10, P 
doi_str_mv 10.1111/ced.12899
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However, only a few studies analysing the clinical features of malignancy‐associated SS have been published in recent years. Aim To retrospectively study the clinical features of SS that could predict the development of associated malignancies and to analyse the development of malignant neoplasia during long‐term follow‐up of patients with SS. Methods Clinical features of the patients diagnosed with SS syndrome between 1987 and 2013 at Bellvitge Hospital (Barcelona, Spain) were retrospectively analysed. Results In total, 138 patients were included in the study (66 male, 72 female, mean ± SD age 51.24 ± 14.11 years). SS was associated with haematological malignancy in 31 cases, infection in 23, inflammatory bowel disease in 12, inflammatory systemic disease in 8, and solid tumours in 4. It was drug‐induced in 5 cases and idiopathic in 54. In four patients, an underlying haematological disease that was considered related to SS was diagnosed between 4 and 16 months after SS presentation. Variables significantly associated with malignancy in multivariate logistic regression analysis were age (OR = 1.08 for each increasing year, P = 0.01), anaemia (OR = 9.38, P = 0.001), thrombocytopenia (OR = 16.10, P &lt; 0.01) and absence of arthralgia (OR = 11.13, P &lt; 0.01). Conclusions Patients with older age, anaemia or thrombocytopenia, and without arthralgia are more likely to have malignancy‐associated SS. We recommend that patients with SS without clear aetiology should be followed up for at least 16 months to exclude a possible underlying haematological malignancy.</description><identifier>ISSN: 0307-6938</identifier><identifier>EISSN: 1365-2230</identifier><identifier>DOI: 10.1111/ced.12899</identifier><identifier>PMID: 27663147</identifier><identifier>CODEN: CEDEDE</identifier><language>eng</language><publisher>England: Blackwell Publishing Ltd</publisher><subject>Adolescent ; Adult ; Aged ; Aged, 80 and over ; Female ; Follow-Up Studies ; Hematologic Neoplasms - etiology ; Hematologic Neoplasms - pathology ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Risk Factors ; Spain ; Sweet Syndrome - complications ; Sweet Syndrome - pathology ; Sweet Syndrome - therapy ; Time Factors ; Young Adult</subject><ispartof>Clinical and experimental dermatology, 2016-10, Vol.41 (7), p.741-746</ispartof><rights>2016 British Association of Dermatologists</rights><rights>2016 British Association of Dermatologists.</rights><rights>Copyright © 2016 British Association of Dermatologists</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3919-4f2faedacd32020eb0d0a5dc4b0433e768ffd5d2fbe7d7d41c9f1f63a593c90f3</citedby><cites>FETCH-LOGICAL-c3919-4f2faedacd32020eb0d0a5dc4b0433e768ffd5d2fbe7d7d41c9f1f63a593c90f3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/27663147$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Marcoval, J.</creatorcontrib><creatorcontrib>Martín-Callizo, C.</creatorcontrib><creatorcontrib>Valentí-Medina, F.</creatorcontrib><creatorcontrib>Bonfill-Ortí, M.</creatorcontrib><creatorcontrib>Martínez-Molina, L.</creatorcontrib><title>Sweet syndrome: long-term follow-up of 138 patients</title><title>Clinical and experimental dermatology</title><addtitle>Clin Exp Dermatol</addtitle><description>Summary Background Several studies support a strong association of Sweet syndrome (SS) with malignancy. However, only a few studies analysing the clinical features of malignancy‐associated SS have been published in recent years. Aim To retrospectively study the clinical features of SS that could predict the development of associated malignancies and to analyse the development of malignant neoplasia during long‐term follow‐up of patients with SS. Methods Clinical features of the patients diagnosed with SS syndrome between 1987 and 2013 at Bellvitge Hospital (Barcelona, Spain) were retrospectively analysed. Results In total, 138 patients were included in the study (66 male, 72 female, mean ± SD age 51.24 ± 14.11 years). SS was associated with haematological malignancy in 31 cases, infection in 23, inflammatory bowel disease in 12, inflammatory systemic disease in 8, and solid tumours in 4. It was drug‐induced in 5 cases and idiopathic in 54. In four patients, an underlying haematological disease that was considered related to SS was diagnosed between 4 and 16 months after SS presentation. Variables significantly associated with malignancy in multivariate logistic regression analysis were age (OR = 1.08 for each increasing year, P = 0.01), anaemia (OR = 9.38, P = 0.001), thrombocytopenia (OR = 16.10, P &lt; 0.01) and absence of arthralgia (OR = 11.13, P &lt; 0.01). Conclusions Patients with older age, anaemia or thrombocytopenia, and without arthralgia are more likely to have malignancy‐associated SS. We recommend that patients with SS without clear aetiology should be followed up for at least 16 months to exclude a possible underlying haematological malignancy.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Female</subject><subject>Follow-Up Studies</subject><subject>Hematologic Neoplasms - etiology</subject><subject>Hematologic Neoplasms - pathology</subject><subject>Humans</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Retrospective Studies</subject><subject>Risk Factors</subject><subject>Spain</subject><subject>Sweet Syndrome - complications</subject><subject>Sweet Syndrome - pathology</subject><subject>Sweet Syndrome - therapy</subject><subject>Time Factors</subject><subject>Young Adult</subject><issn>0307-6938</issn><issn>1365-2230</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2016</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp10MtKxDAUBuAgio6XhS8gBTe6qCY5bdK6k3G8oCjibRkyzYlU22ZMWsZ5ezuO40Iwm0D4zk_OT8guo0esP8cFmiPGszxfIQMGIo05B7pKBhSojEUO2QbZDOGNUgZMputkg0shgCVyQOBhithGYdYY72o8iSrXvMYt-jqyrqrcNO4mkbMRgyya6LbEpg3bZM3qKuDOz71Fns5Hj8PL-Obu4mp4ehMXkLM8Tiy3Go0uDHDKKY6poTo1RTKmCQBKkVlrUsPtGKWRJmFFbpkVoNMcipxa2CIHi9yJdx8dhlbVZSiwqnSDrguKZf2aIKRIerr_h765zjf97-aKpwln2VwdLlThXQgerZr4stZ-phhV8yZV36T6brK3ez-J3bjuX5dyWV0PjhdgWlY4-z9JDUdny8h4MVGGFj9_J7R_V0KCTNXL7YVi188ye75-UPfwBciAikA</recordid><startdate>201610</startdate><enddate>201610</enddate><creator>Marcoval, J.</creator><creator>Martín-Callizo, C.</creator><creator>Valentí-Medina, F.</creator><creator>Bonfill-Ortí, M.</creator><creator>Martínez-Molina, L.</creator><general>Blackwell Publishing Ltd</general><general>Oxford University Press</general><scope>BSCLL</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>K9.</scope><scope>7X8</scope></search><sort><creationdate>201610</creationdate><title>Sweet syndrome: long-term follow-up of 138 patients</title><author>Marcoval, J. ; Martín-Callizo, C. ; Valentí-Medina, F. ; Bonfill-Ortí, M. ; Martínez-Molina, L.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3919-4f2faedacd32020eb0d0a5dc4b0433e768ffd5d2fbe7d7d41c9f1f63a593c90f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2016</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Aged</topic><topic>Aged, 80 and over</topic><topic>Female</topic><topic>Follow-Up Studies</topic><topic>Hematologic Neoplasms - etiology</topic><topic>Hematologic Neoplasms - pathology</topic><topic>Humans</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Retrospective Studies</topic><topic>Risk Factors</topic><topic>Spain</topic><topic>Sweet Syndrome - complications</topic><topic>Sweet Syndrome - pathology</topic><topic>Sweet Syndrome - therapy</topic><topic>Time Factors</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Marcoval, J.</creatorcontrib><creatorcontrib>Martín-Callizo, C.</creatorcontrib><creatorcontrib>Valentí-Medina, F.</creatorcontrib><creatorcontrib>Bonfill-Ortí, M.</creatorcontrib><creatorcontrib>Martínez-Molina, L.</creatorcontrib><collection>Istex</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health &amp; Medical Complete (Alumni)</collection><collection>MEDLINE - Academic</collection><jtitle>Clinical and experimental dermatology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Marcoval, J.</au><au>Martín-Callizo, C.</au><au>Valentí-Medina, F.</au><au>Bonfill-Ortí, M.</au><au>Martínez-Molina, L.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Sweet syndrome: long-term follow-up of 138 patients</atitle><jtitle>Clinical and experimental dermatology</jtitle><addtitle>Clin Exp Dermatol</addtitle><date>2016-10</date><risdate>2016</risdate><volume>41</volume><issue>7</issue><spage>741</spage><epage>746</epage><pages>741-746</pages><issn>0307-6938</issn><eissn>1365-2230</eissn><coden>CEDEDE</coden><abstract>Summary Background Several studies support a strong association of Sweet syndrome (SS) with malignancy. However, only a few studies analysing the clinical features of malignancy‐associated SS have been published in recent years. Aim To retrospectively study the clinical features of SS that could predict the development of associated malignancies and to analyse the development of malignant neoplasia during long‐term follow‐up of patients with SS. Methods Clinical features of the patients diagnosed with SS syndrome between 1987 and 2013 at Bellvitge Hospital (Barcelona, Spain) were retrospectively analysed. Results In total, 138 patients were included in the study (66 male, 72 female, mean ± SD age 51.24 ± 14.11 years). SS was associated with haematological malignancy in 31 cases, infection in 23, inflammatory bowel disease in 12, inflammatory systemic disease in 8, and solid tumours in 4. It was drug‐induced in 5 cases and idiopathic in 54. In four patients, an underlying haematological disease that was considered related to SS was diagnosed between 4 and 16 months after SS presentation. Variables significantly associated with malignancy in multivariate logistic regression analysis were age (OR = 1.08 for each increasing year, P = 0.01), anaemia (OR = 9.38, P = 0.001), thrombocytopenia (OR = 16.10, P &lt; 0.01) and absence of arthralgia (OR = 11.13, P &lt; 0.01). Conclusions Patients with older age, anaemia or thrombocytopenia, and without arthralgia are more likely to have malignancy‐associated SS. We recommend that patients with SS without clear aetiology should be followed up for at least 16 months to exclude a possible underlying haematological malignancy.</abstract><cop>England</cop><pub>Blackwell Publishing Ltd</pub><pmid>27663147</pmid><doi>10.1111/ced.12899</doi><tpages>6</tpages></addata></record>
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source MEDLINE; Oxford University Press Journals All Titles (1996-Current); Alma/SFX Local Collection
subjects Adolescent
Adult
Aged
Aged, 80 and over
Female
Follow-Up Studies
Hematologic Neoplasms - etiology
Hematologic Neoplasms - pathology
Humans
Male
Middle Aged
Retrospective Studies
Risk Factors
Spain
Sweet Syndrome - complications
Sweet Syndrome - pathology
Sweet Syndrome - therapy
Time Factors
Young Adult
title Sweet syndrome: long-term follow-up of 138 patients
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