诊断病例

Case for diagnosis.

作者信息

Lima Lourenço de Azevedo, Haddad Natacha de Carvalho Mello, Lima Ricardo Barbosa, D'Acri Antonio Macedo, Martins Carlos José

机构信息

Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.

出版信息

An Bras Dermatol. 2014 May-Jun;89(3):521-2. doi: 10.1590/abd1806-4841.20142863.

DOI:10.1590/abd1806-4841.20142863

PMID:24937836

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4056720/

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.

摘要

迪戈斯病，又称恶性萎缩性丘疹病，是一种病因不明的罕见闭塞性血管病，其特征为真皮、胃肠道、中枢神经系统及其他器官出现梗死。其特点为丘疹，丘疹会形成脐凹，并演变为中央呈凹陷瓷白色区域，周围有带毛细血管扩张的红斑晕。组织学表现包括楔形真皮表皮坏死和血管血栓形成。约50 - 60%有全身症状的患者会在2 - 3年内死亡，多数死于胃肠道穿孔。我们报告一例45岁女性的典型病例，该病例为致死性结局。