Intestine Transplantation in Degos Disease (Malignant Atrophic Papulosis): Report of a Case with focus on Clinic and Histology as chronologically evolved.

摘要

We describe a 30-year-old woman with suspect Degos disease who underwent intestine transplantation after small-bowel infarction due to mesenteric venous thrombosis. Patient was submitted 5 months earlier to colon resection for perforation; she showed even multiple skin lesions, consistent with those found in Degos disease. A multivisceral transplant was performed at our Hospital in May 2008. Immunosuppres-sion was done according to standard protocols. Scheduling intestinal biopsies were performed, and these were always negative for rejection or infection. Two months after transplant, patient developed many more skin lesions; skin biopsies were performed, confirming the diagnosis of Degos disease. Patient died 4 months after transplant for the onset of systemic cytomegalovirus infection and multiorgan failure. Post-mortem examination confirmed the histological nature of the skin lesions and the systemic cytomegalovirus infection; no cerebral lesions were observed.