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

[malignant atrophic papulosis]

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 .