[Long-term survival in malignant atrophic papulosis: a case report and review of the Japanese literature].

Malignant atrophic papulosis (MAP) is characterized by skin lesion and high mortality rate caused by perforation of the GI tract or involvement of the central nervous system. Approximately 100 cases have been reported in the world literatures, but etiology is unknown. A 44-year-old female patient was admitted for chest and abdominal pain with characteristic papuloses, which had been noted by the patient seven years ago. Because of positive peritoneal irritation and intraabdominal free air, exploratory laparotomy was done. Multiple red inflammatory or yellow atrophic maculae on the entire intestine with no obvious perforation but with air-leak were found. Those perforations were closed with seromuscular sutures. The patient is doing well sixteen months after surgery. Fourteen MAP cases have been reported in the Japanese literature. As is found in the world literature, the mortality rate is extremely high. All of reported cases were initially diagnosed because of the particular skin lesions. Abdominal symptoms developed in 10 cases and six of these died. Three cases died within three weeks after bowel resection. There are three surviving cases. One was treated conservatively even though intraabdominal free air was present. Two had three operations, including one simple closure and two intraabdominal explorations. According to this result, a bowel resection should not be performed on MAP patients because of this high mortality. Administration of anticoagulants, i.e., heparin, prostaglandin E1 and ticlopidine seems to be effective in alleviating symptoms and might prevent further deterioration.