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Degos disease, also known as malignant atrophic papulosis, is a rare occlusive vasculopathy of unknown etiology characterized by infarcts in the dermis, gastrointestinal tract, central nervous system, and other organs. It is characterized by papules, which become umbilicated and evolve with a depres...

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Detalles Bibliográficos
Autores principales: Lima, Lourenço de Azevedo, Haddad, Natacha de Carvalho Mello, Lima, Ricardo Barbosa, D'Acri, Antonio Macedo, Martins, Carlos José
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Sociedade Brasileira de Dermatologia 2014
Materias:
What Is Your Diagnosis?
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4056720/
https://www.ncbi.nlm.nih.gov/pubmed/24937836
http://dx.doi.org/10.1590/abd1806-4841.20142863
Descripción
Sumario:Degos disease, also known as malignant atrophic papulosis, is a rare occlusive vasculopathy of unknown etiology characterized by infarcts in the dermis, gastrointestinal tract, central nervous system, and other organs. It is characterized by papules, which become umbilicated and evolve with a depressed porcelain-white central area, with an erythematous halo with telangiectasias. Histological findings include wedge-shaped dermoepidermal necrosis and blood vessel thrombosis. Approximately 50-60% of patients with systemic symptoms die within 2-3 years, most due to gastrointestinal perforation. We report a typical case, with lethal outcome, in a 45-year-old woman.

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