{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T09:39:18Z","timestamp":1789810758133,"version":"4.0.1"},"reference-count":129,"publisher":"MDPI AG","issue":"22","license":[{"start":{"date-parts":[[2022,11,21]],"date-time":"2022-11-21T00:00:00Z","timestamp":1668988800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Medical University of Lublin","award":["PBmb164"],"award-info":[{"award-number":["PBmb164"]}]},{"name":"Medical University of Lublin","award":["DS640"],"award-info":[{"award-number":["DS640"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Lymphomatoid papulosis (LyP) is a very rare disease that belongs to the group of CD30+ lymphoproliferative skin diseases. LyP is localized or generalized and usually presents as isolated or clustered red\/brown-red lesions in the form of nodules and\/or papules. The course of the disease is in most cases mild; however, depending on concomitant risk factors and history, it may progress to lymphoma, significantly reducing the survival rate and prognosis. Importantly, the clinical picture of the disease remains somewhat ambiguous, leading to a large number of misdiagnoses that result in inappropriate treatment, which is usually insufficient to alleviate symptoms. In addition to clinical manifestations, the histological characteristics vary widely and usually overlap with other conditions, especially those belonging to the group of lymphoproliferative disorders. Although diagnosis remains a challenge, several recommendations and guidelines have been introduced to standardize and facilitate the diagnostic process. This article reviews the available literature on the most important aspects of etiopathogenesis, clinical and histopathological features, diagnostic criteria, and possible treatment strategies for LyP, with particular emphasis on the role of the immune system.<\/jats:p>","DOI":"10.3390\/cells11223697","type":"journal-article","created":{"date-parts":[[2022,11,22]],"date-time":"2022-11-22T03:08:37Z","timestamp":1669086517000},"page":"3697","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Etiopathogenesis, Diagnosis, and Treatment Strategies for Lymphomatoid Papulosis with Particular Emphasis on the Role of the Immune System"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1717-4280","authenticated-orcid":false,"given":"Danuta","family":"Nowicka","sequence":"first","affiliation":[{"name":"Department of Dermatology, Venereology and Allergology, Wroc\u0142aw Medical University, 50-368 Wroc\u0142aw, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9530-6659","authenticated-orcid":false,"given":"Paulina","family":"Mertowska","sequence":"additional","affiliation":[{"name":"Department of Experimental Immunology, Medical University of Lublin, 20-093 Lublin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7121-1396","authenticated-orcid":false,"given":"Sebastian","family":"Mertowski","sequence":"additional","affiliation":[{"name":"Department of Experimental Immunology, Medical University of Lublin, 20-093 Lublin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Hymos","sequence":"additional","affiliation":[{"name":"Department of Experimental Immunology, Medical University of Lublin, 20-093 Lublin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8714-7627","authenticated-orcid":false,"given":"Alicja","family":"Forma","sequence":"additional","affiliation":[{"name":"Department of Forensic Medicine, Medical University of Lublin, 20-090 Lublin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9598-373X","authenticated-orcid":false,"given":"Adam","family":"Michalski","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology and Immunotherapy, Medical University of Lublin, 20-093 Lublin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9462-8779","authenticated-orcid":false,"given":"Izabela","family":"Morawska","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology and Immunotherapy, Medical University of Lublin, 20-093 Lublin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0688-6928","authenticated-orcid":false,"given":"Rafa\u0142","family":"Hrynkiewicz","sequence":"additional","affiliation":[{"name":"Institute of Biology, University of Szczecin, 71-412 Szczecin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4065-3842","authenticated-orcid":false,"given":"Paulina","family":"Nied\u017awiedzka-Rystwej","sequence":"additional","affiliation":[{"name":"Institute of Biology, University of Szczecin, 71-412 Szczecin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0451-4741","authenticated-orcid":false,"given":"Ewelina","family":"Grywalska","sequence":"additional","affiliation":[{"name":"Department of Experimental Immunology, Medical University of Lublin, 20-093 Lublin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,11,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1111\/j.1610-0387.2007.06337.x","article-title":"Cutaneous lymphomas in Germany: An analysis of the Central Cutaneous Lymphoma Registry of the German Society of Dermatology (DDG)","volume":"5","author":"Assaf","year":"2007","journal-title":"JDDG J. 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