[Köhlmeier-Degos disease (Malignant Atrophic Papulosis): a cause of recurrent multiple intestinal perforations]

Abstract

Malignant Atrophic Papulosis (MAP) or Degos disease is a very rare vasculopathy affecting multiple systems. When associated with gastrointestinal tract or central nervous system involvment, patients with Degos disease have a poor prognosis and a high mortality. We report a case of Degos disease with systemic involvement, which ultimately caused peritonitis, sepsis, and death, despite all treatment. A 29-year-old man was admitted in June 2007 on the surgical ward of our hospital with acute generalized abdominal pain and multiple skin lesions. The patient had an appendectomy at another hospital 6 weeks before the current presentation. The exploratory laparotomy showed 2 perforations of the ileum. After the initial procedure, the patient developed spontaneous recurrent perforations of the small bowel and suffered another 3 reoperations. Neuroradiologic reports described central and peripheral nervous system involvement with progressive clinical deterioration and a meningovascular pattern at cerebral MRI. Despite aggressive treatment the patient died 3 months after the first surgical intervention.