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- W4366088052 abstract "Background: Atrophic papulosis (Köhlmeier-Degos disease, Degos disease) is a rare thrombo-obliterative microangiopathy of unknown pathogenesis. It usually affects people between the ages of 20 and 50. The disease is considered uncommon in children. Objective: Clinical characterization of pediatric patients with atrophic papulosis (pAP). Methods: Single-center prospective cohort study with data derived from the international Degos Disease Registry collected between 2000 and 2021. Cases were referred from different centers worldwide to the evaluation center in Germany (Berlin and Dessau). The questionnaires and medical records provided were critically evaluated. Results: Among 96 patients fulfilling the criteria, 19 were aged 0 to completed 17 years at the time of onset. The median age at the onset was 5 years, ranging from 0-1 year for girls to 8 years for boys. In contrast to adult patients (male-to-female ratio 1:2.2), there was a male predominance in pediatric patients with a male-to-female ratio of 1.7:1. Systemic involvement, in particular gastrointestinal, central nervous system and cardiac, was more frequent in children than in adults. There were no statistically significant differences between the two groups' family history, multisystem involvement, mortality and median survival time. Conclusions: This study represents the first original analysis of a pAP cohort. Atrophic papulosis has some distinct features in the pediatric population. Paying attention to the typical skin lesions in combination with neurological or gastrointestinal symptoms is mandatory to make a prompt and accurate diagnosis." @default.
- W4366088052 created "2023-04-19" @default.
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- W4366088052 date "2023-05-01" @default.
- W4366088052 modified "2023-10-16" @default.
- W4366088052 title "398 Atrophic papulosis (Köhlmeier-Degos disease) in children and adolescents - a cross–sectional study" @default.
- W4366088052 doi "https://doi.org/10.1016/j.jid.2023.03.404" @default.
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