{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T00:46:47Z","timestamp":1791334007117,"version":"4.3.1"},"reference-count":14,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2022,7,8]],"date-time":"2022-07-08T00:00:00Z","timestamp":1657238400000},"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":[[2022,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Although the merely cutaneous, benign form of the extremely rare disease atrophic papulosis (K\u00f6hlmeier\u2013Degos disease) may occasionally develop into the systemic, malignant form with time, it is unclear whether it exhibits any systemic characteristics.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To determine whether benign atrophic papulosis exhibits inflammatory and thrombo\u2010occlusive signals and to classify it according to the Chapel\u2010Hill classification of vasculitis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In a monocentric, controlled study, levels of cytokines (IL\u20101\u03b2, IL\u20106, IL\u20108, IFN\u03b3, MCP\u20101, VEGF, TNF\u03b1, TGF\u2010\u03b21), antiphospholipid antibodies (cardiolipin IgG\/A\/M, cardiolipin IgG, cardiolipin IgM, \u03b22\u2010glycoprotein IgG\/A\/M, phosphatidyl choline, phosphatidyl serine, phosphatidyl inositol, phosphatidyl ethanolamine and sphingomyelin A), antibodies against proteinase\u20103 IgG and myeloperoxidase IgG, antinuclear antibodies and extractable nuclear antigen were assessed in blood samples of six benign atrophic papulosis patients and six age\u2010 and sex\u2010matched healthy controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>IL\u20108 was only detectable in patients' serum. VEGF was reduced and cardiolipin IgG\/A\/M and \u03b22\u2010glycoprotein antibodies were increased in the patients' group. ANA were only detected in three patients, and ENA were negative throughout. No differences were detected between the other investigated markers.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This is the first study evaluating systemic inflammatory and thrombo\u2010occlusive vessel signalling in benign atrophic papulosis and provides evidence of a non\u2010antineutrophil cytoplasmatic antibodies immune\u2010complex small vessel vasculitis according to the Chapel\u2010Hill classification. These findings corroborate its systemic character despite the apparent missing involvement of systemic organs.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jdv.18362","type":"journal-article","created":{"date-parts":[[2022,6,24]],"date-time":"2022-06-24T07:49:07Z","timestamp":1656056947000},"page":"2195-2198","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Inflammation and thrombo\u2010occlusive vessel signalling in benign atrophic papulosis (<scp>K\u00f6hlmeier\u2013Degos<\/scp> disease)"],"prefix":"10.1111","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1646-2608","authenticated-orcid":false,"given":"C.C.","family":"Zouboulis","sequence":"first","affiliation":[{"name":"Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center Brandenburg Medical School Theodor Fontane and Faculty of Health Sciences Brandenburg  Dessau Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Theodoridis","sequence":"additional","affiliation":[{"name":"Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center Brandenburg Medical School Theodor Fontane and Faculty of Health Sciences Brandenburg  Dessau Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Makrantonaki","sequence":"additional","affiliation":[{"name":"Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center Brandenburg Medical School Theodor Fontane and Faculty of Health Sciences Brandenburg  Dessau Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,7,8]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-10"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.12642"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-021-01819-z"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1309\/AJCP66QIMFARLZKI"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2017.05.024"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2020.102716"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-06-2416"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2019.100610"},{"key":"e_1_2_8_10_1","first-page":"724592","article-title":"Detection of antiphosphatidylserine\/prothrombin antibodies and their potential diagnostic value","volume":"2013","author":"Cigon P","year":"2013","journal-title":"Clin Develop Immunol"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000372-200308000-00005"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2012.08.002"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.17377"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.14355"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-022-02322-9"}],"container-title":["Journal of the European Academy of Dermatology and Venereology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jdv.18362","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/jdv.18362","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jdv.18362","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,20]],"date-time":"2023-08-20T19:05:35Z","timestamp":1692558335000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/jdv.18362"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,7,8]]},"references-count":14,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2022,11]]}},"alternative-id":["10.1111\/jdv.18362"],"URL":"https:\/\/doi.org\/10.1111\/jdv.18362","archive":["Portico"],"relation":{},"ISSN":["0926-9959","1468-3083"],"issn-type":[{"value":"0926-9959","type":"print"},{"value":"1468-3083","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,7,8]]},"assertion":[{"value":"2022-03-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-06-03","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-07-08","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}