Papulose atrophiante bénigne : maladie de Degos revisitée

Modiano, P.; Guern, A. Le; Lebas, D.; Lasek, A.; Lamiaux, Marie

doi:10.1016/j.annder.2017.09.504

Cited by 1 publication

(2 citation statements)

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“…The available data on pAP mainly derives from case reports and, interestingly, from articles mostly published in neurological journals (Table 1). 3,6,9–41 Atrophic papulosis is considered to affect middle‐aged adults. However, a substantial number of patients in our cross–sectional study were children (20%; 19/96).…”

Section: Discussionmentioning

confidence: 99%

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Atrophic papulosis (Köhlmeier–Degos disease) in children and adolescents—A cross–sectional study and literature review

Zouboulis

Kaleta

Broniatowska

et al. 2023

Acad Dermatol Venereol

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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. However, it can occur at any age. The condition is considered uncommon in children. Objective: Clinical characterization of paediatric patients with atrophic papulosis. Methods: Single-centre prospective cohort study with data derived from the international Degos Disease Registry collected between 2000 and 2021.Results: Among 96 registered patients with atrophic papulosis fulfilling the criteria, 19 were aged 0 to completed 17 years at the time of onset. The median age at the time of onset was 5 years, ranging from 0 to 1 years 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 paediatric 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 adult patients. There were no statistically significant differences between family history, multisystem involvement, mortality and median survival time in the two groups. Conclusions: Atrophic papulosis has some distinct features in the paediatric population. It presents an important and still under-recognized 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.How to cite this article: Zouboulis CC, Kaleta KP, Broniatowska E, Jarienė V, Nikolakis G. Atrophic papulosis (Köhlmeier-Degos disease) in children and adolescents-A cross-sectional study and literature review.

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Section: Discussionmentioning

confidence: 99%

“…However, the number of case reports of paediatric atrophic papulosis (pAP) is constantly growing (Table 1). 3,6,9–41 Our study aimed to investigate the early occurrence of the disease, including congenital manifestations, and to compare this to adult atrophic papulosis (aAP).…”

Section: Introductionmentioning

confidence: 99%