Degos' disease mimicking vasculitis.

Introduction Degos’ disease is a rare disorder with multisystem involvement and unknown etiology. This entity was first described by Degos in 1942 (1,2). Other synonyms for this disease are malignant atrophic papulosis, atrophic papulosquamous dermatitis, fatal cutaneous-intestinal syndrome, and thromboangiitis cutaneointestinalis disseminata (3). It has been more commonly reported in whites, men, and those in the third decade of life, although onset age ranges from 3 weeks to 67 years (4). The average course of the disease is reported to be around 2 years (1), but case reports of patients with a benign variant have been reported with survival of 14 years (5). Death is most commonly due to intestinal perforation or cerebral infarction. We report a fatal case of Degos’ disease that presented with abdominal pain and intestinal perforations, which clinically mimicked vasculitis.