Presentation of Degos Syndrome as Acute Small-Bowel Perforation

Malignant atrophic papulosis is an uncommon vasculopathy affecting multiple systems and frequently associated with a poor prognosis. We describe a 56-year-old patient with this rare disorder whose death was the result of intestinal involvement. Clinical diagnostic features of malignant atrophic papulosis as well as the main therapeutic approaches suggested in the literature are presented herein.