{"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":1791334007764,"version":"4.3.1"},"reference-count":22,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2022,6,7]],"date-time":"2022-06-07T00:00:00Z","timestamp":1654560000000},"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>Atrophic papulosis is a very rare vascular disease of unknown pathogenesis, mostly described by case reports.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To assess demographic data and prognosis in patients with atrophic papulosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A single\u2010centre study was performed on a series of 105 patients with atrophic papulosis, diagnosed 2000\u20132021. Patients were referred and diagnosed at the evaluation centre and patients' clinical data were provided by the Degos Support Network and evaluated by the authors for confirming the diagnosis of skin lesions and fulfilling the diagnostic criteria for a malignant subset. A unique set of variables were collected from all patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The mean age of disease onset was 33.3\u2009\u00b1\u200918.3\u2009years and the male\u2010to\u2010female ratio was 1:1.6. The family history rate was 8.1%. The classification into a benign, merely cutaneous disease (benign atrophic papulosis), and malignant atrophic papulosis, associating cutaneous and visceral lesions was confirmed due to their striking prognostic difference. Benign atrophic papulosis was detected in 41% of the patients with no deaths occurring throughout the follow\u2010up period (median 3.00\u2009years; range 0.13\u201323). Malignant atrophic papulosis was reported in 59% of patients with 47.5% multisystemic involvement and a median skin lesion onset to systemic symptoms duration of 0.54\u2009years (\u22126 to 20). The gastrointestinal tract and central nervous system were equally involved; however, the neurological involvement\u2010caused death rate was slightly higher. The disease\u2010specific mortality rate of malignant atrophic papulosis was 22.6%.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Atrophic papulosis presents with a striking prognostic difference of benign \u2013 merely cutaneous \u2013 involvement or quickly developing \u2013 into less than 1 year \u2013 malignant subset, associating cutaneous and visceral lesions and multiorgan involvement in 1\/2 of the patients, which leads to premature, disease\u2010specific mortality in 1\/4 of the cases. Central nervous system and gastrointestinal tract complications are the major reasons for disease\u2010specific death. Over the years, the diagnosis of severe nervous system involvement has become more common.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jdv.18260","type":"journal-article","created":{"date-parts":[[2022,5,25]],"date-time":"2022-05-25T04:23:19Z","timestamp":1653452599000},"page":"2190-2194","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Atrophic papulosis (<scp>K\u00f6hlmeier\u2013Degos<\/scp> disease) revisited: a cross\u2010sectional study on 105 patients"],"prefix":"10.1111","volume":"36","author":[{"given":"K.P.","family":"Kaleta","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"},{"name":"Department of Dermatology Jagiellonian University Medical College  Krakow Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V.","family":"Jarien\u0117","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"},{"name":"Department of Skin and Venereal Diseases, Lithuanian University of Health Sciences (LUHS) Hospital of LUHS Kauno Klinikos  Kaunas Lithuania"}],"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":"G.","family":"Nikolakis","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"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1646-2608","authenticated-orcid":false,"given":"C.C.","family":"Zouboulis","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":[[2022,6,7]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01828450"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-10"},{"key":"e_1_2_8_4_1","first-page":"148","article-title":"Dermatite papulosqameuse atrophiante","volume":"49","author":"Degos R","year":"1942","journal-title":"Bull Soc Fr Dermatol Syphiligr"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.12642"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-021-01819-z"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.2002.04677.x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMcpc069029"},{"key":"e_1_2_8_9_1","first-page":"298","article-title":"Papulosis maligna atrophicans (Morbus K\u00f6hlmeyer\u2010Degos)","volume":"76","author":"K\u00f6lmel K","year":"2001","journal-title":"Z Hautkr"},{"key":"e_1_2_8_10_1","first-page":"1","article-title":"Sindrome cutaneo\u2010mucoso\u2010intestinale; 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