Degos' disease (malignant atrophic papulosis) as a fatal cause of acute abdomen: report of a case

Abstract

Degos' disease, otherwise known as "malignant atrophic papulosis," is a rare condition characterized by typical cutaneous lesions. Its involvement of the gastrointestinal (GI) tract is usually associated with a poor prognosis. We report a case of Degos' disease with GI involvement, which ultimately caused peritonitis, sepsis, and death, despite all treatment measures. A 59-year old woman was admitted to our hospital with acute generalized abdominal pain. The patient had presented initially with multiple skin lesions 2 years earlier, and even with surgery for small-bowel perforation 10 months before this admission, Degos' disease had not been diagnosed. Explorative laparotomy revealed multifocal, ischemic changes in the small bowel with perforation in the mid-jejunum. After the operation, she suffered recurrent small-bowel fistulas and died within 3 months. In a patient with acute abdominal pain and typical atrophic papules, clinicians should retain a high index of suspicion for Degos' disease with GI involvement, even though it is rare.