title: Case for diagnosis.
abstract: 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.
authors.affiliation: Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
authors.forename: Lourenço de Azevedo
authors.initials: Lde A
authors.lastname: Lima
authors.affiliation: Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
authors.forename: Natacha de Carvalho Mello
authors.initials: Nde C
authors.lastname: Haddad
authors.affiliation: Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
authors.forename: Ricardo Barbosa
authors.initials: RB
authors.lastname: Lima
authors.affiliation: Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
authors.forename: Antonio Macedo
authors.initials: AM
authors.lastname: D'Acri
authors.affiliation: Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
authors.forename: Carlos José
authors.initials: CJ
authors.lastname: Martins
mesh_terms: D001706:Biopsy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007416:Intestinal Perforation / Q000150:complications; D054853:Malignant Atrophic Papulosis / Q000150:complications / Q000473:pathology*; D008875:Middle Aged; D012867:Skin / Q000473:pathology*; D020246:Venous Thrombosis / Q000473:pathology