{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T20:11:18Z","timestamp":1783023078025,"version":"3.54.6"},"reference-count":31,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2020,3,10]],"date-time":"2020-03-10T00:00:00Z","timestamp":1583798400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acad Dermatol Venereol"],"published-print":{"date-parts":[[2020,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive type of haematologic precursor malignancy primarily often manifesting in the skin. We sought to provide a thorough clinical characterization and report our experience on therapeutic approaches to <jats:styled-content style=\"fixed-case\">BPDCN<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In the present multicentric retrospective study, we collected all <jats:styled-content style=\"fixed-case\">BPDCN<\/jats:styled-content> cases occurring between 05\/1999 and 03\/2018 in 10 secondary care centres of the German\u2013Swiss\u2013Austrian cutaneous lymphoma working group.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 37 <jats:styled-content style=\"fixed-case\">BPDCN<\/jats:styled-content> cases were identified and included. Almost 90% of the patients had systemic manifestations (bone marrow, lymph nodes, peripheral blood) in addition to skin involvement. The latter presented with various types of cutaneous lesions: nodular (in more than 2\/3) and bruise\u2010like (in 1\/3) skin lesions, but also maculopapular exanthema (in circa 1\/6). Therapeutically, 22 patients received diverse combinations of chemotherapeutic regimens and\/or radiotherapy. Despite initial responses, all of them ultimately relapsed and died from progressive disease. Eleven patients underwent haematopoietic stem cell transplantation (<jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>; autologous <jats:styled-content style=\"fixed-case\">HSCT <\/jats:styled-content><jats:italic>n<\/jats:italic>\u00a0=\u00a03, allo\u2010<jats:styled-content style=\"fixed-case\">HSCT <\/jats:styled-content><jats:italic>n<\/jats:italic>\u00a0=\u00a08). The mortality rate among <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content> patients was only 33.33% with a median survival time of 60.5\u00a0months.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our study demonstrates the clinical diversity of cutaneous <jats:styled-content style=\"fixed-case\">BPDCN<\/jats:styled-content> manifestations and the positive development observed after the introduction of <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jdv.16215","type":"journal-article","created":{"date-parts":[[2020,1,19]],"date-time":"2020-01-19T10:49:41Z","timestamp":1579430981000},"page":"1489-1495","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Clinical diversity and treatment approaches to blastic plasmacytoid dendritic cell neoplasm: a retrospective multicentre study"],"prefix":"10.1111","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8607-6254","authenticated-orcid":false,"given":"M.\u2010C.","family":"Br\u00fcggen","sequence":"first","affiliation":[{"name":"Department of Dermatology University Hospital of Zurich Zurich Switzerland"},{"name":"Faculty of Medicine University of Zurich Zurich Switzerland"},{"name":"Medical Campus Davos Davos Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4737-2920","authenticated-orcid":false,"given":"J.","family":"Valencak","sequence":"additional","affiliation":[{"name":"Department of Dermatology Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1882-4908","authenticated-orcid":false,"given":"R.","family":"Stranzenbach","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology, Allergology and Phlebology Johannes Wesling Medical Centre University Hospital of Ruhr\u2010University Bochum Minden Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Dermatology University Hospital of Zurich Zurich Switzerland"},{"name":"Faculty of Medicine University of Zurich Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2683-6028","authenticated-orcid":false,"given":"R.","family":"Stadler","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology, Allergology and Phlebology Johannes Wesling Medical Centre University Hospital of Ruhr\u2010University Bochum Minden Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Jonak","sequence":"additional","affiliation":[{"name":"Department of Dermatology Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W.","family":"Bauer","sequence":"additional","affiliation":[{"name":"Department of Dermatology Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Porkert","sequence":"additional","affiliation":[{"name":"Department of Dermatology Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Blaschke","sequence":"additional","affiliation":[{"name":"Department of Dermatology Medical University of Vienna Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4044-9094","authenticated-orcid":false,"given":"F.","family":"Meiss","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venereology Faculty of Medicine University Medical Center \u2013 University of Freiburg Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0220-0551","authenticated-orcid":false,"given":"J. 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