A fatal case of Degos' disease which presented with recurrent intestinal perforation  

作　　者：Mona Ahmadi Sevil Agabalaey Rafi Zhale Faham Ramin Azhough Samad Beheshty Rooy Omid Rahmani 

机构地区：[1]Department of General Surgery and Pathology,Imam Reza Hospital

出　　处：《World Journal of Gastrointestinal Surgery》2011年第10期156-158,共3页世界胃肠外科杂志（英文版）（电子版）

摘　　要：Degos' disease,otherwise known as "malignant atrophic papulosis" is a rare vasculopathy with an unknown etiology characterized by typical cutaneous lesions.Involvement of the gastrointestinal(GI) tract is observed in approximately half of patients and small infarctions in the mucosa can cause perforation and resulting peritonitis,the leading cause of death.We present a fatal case of Degos' disease with skin and GI involvement,manifesting as recurrent intestinal perforations and peritonitis,in a 15-year-old Iranian boy.Degos’ disease, otherwise known as “malignant atrophic papulosis” is a rare vasculopathy with an unknown etiology characterized by typical cutaneous lesions. Involvement of the gastrointestinal (GI) tract is observed in approximately half of patients and small infarctions in the mucosa can cause perforation and resulting peritonitis, the leading cause of death. We present a fatal case of Degos’ disease with skin and GI involvement, manifesting as recurrent intestinal perforations and peritonitis, in a 15-year-old Iranian boy.

关 键 词：Degos’disease Malignant ATROPHIC PAPULOSIS Small BOWEL PERFORATION 

分 类 号：R758.6[医药卫生—皮肤病学与性病学]