The Authors report an exceptional case of Atrophic Malignant Papulosis (P.A.M.) or Degos disease, presenting as acute abdomen with ileal and multiple colic perforations. The disease is due to an occlusive, progressive, thrombotic vascular pathology affecting different parts of the body and may bring to death. Usually this rare disease presents with typical multiple papulous skin lesions, followed by a systemic involvement of the digestive, nervous, and visive tracts. Peritonitis and cerebral infarction are the most frequent causes of death.