{"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":1791334007751,"version":"4.3.1"},"reference-count":50,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,1,14]],"date-time":"2023-01-14T00:00:00Z","timestamp":1673654400000},"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":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Atrophic papulosis (K\u00f6hlmeier\u2013Degos disease, Degos disease) is a rare thrombo\u2010obliterative microangiopathy of unknown pathogenesis. It usually affects people between the ages of 20 and 50. However, it can occur at any age. The condition is considered uncommon in children.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>Clinical characterization of paediatric patients with atrophic papulosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Single\u2010centre prospective cohort study with data derived from the international Degos Disease Registry collected between 2000 and 2021.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Among 96 registered patients with atrophic papulosis fulfilling the criteria, 19 were aged 0 to completed 17\u2009years at the time of onset. The median age at the time of onset was 5\u2009years, ranging from 0 to 1\u2009years for girls to 8\u2009years for boys. In contrast to adult patients (male\u2010to\u2010female ratio 1:2.2), there was a male predominance in paediatric patients with a male\u2010to\u2010female ratio of 1.7:1. Systemic involvement, in particular gastrointestinal, central nervous system and cardiac, was more frequent in children than in adult patients. There were no statistically significant differences between family history, multisystem involvement, mortality and median survival time in the two groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Atrophic papulosis has some distinct features in the paediatric population. It presents an important and still under\u2010recognized problem. Therefore, it is mandatory to pay attention to the typical skin lesions in combination with neurological or gastrointestinal symptoms in order to make a prompt and accurate diagnosis.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jdv.18844","type":"journal-article","created":{"date-parts":[[2023,1,6]],"date-time":"2023-01-06T10:30:46Z","timestamp":1673001046000},"page":"810-816","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Atrophic papulosis (K\u00f6hlmeier\u2013Degos disease) in children and adolescents\u2014A cross\u2013sectional study and literature review"],"prefix":"10.1111","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1646-2608","authenticated-orcid":false,"given":"Christos C.","family":"Zouboulis","sequence":"first","affiliation":[{"name":"Departments of Dermatology, Venereology, Allergology and Immunology, Staedtisches Klinikum Dessau Brandenburg Medical School Theodor Fontane and Faculty of 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