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

[malignant atrophic papulosis]

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 .