Idiopathic pylephlebitis and idiopathic sclerosing peritonitis in a man with protein S deficiency.

Idiopathic pylephlebitis and primary sclerosing peritonitis are two highly unusual entities. To our knowledge, the association of the two diseases has not been described previously. We report a 42-year-old patient with a protein S deficiency who presented with fever and chills, in whom idiopathic pylephlebitis was diagnosed. A year later, the patient was readmitted because of recurrent vomiting and weight loss. An exploratory laparotomy yielded diagnosis of sclerosing peritonitis, which resolved after surgery. The short time interval between the processes suggests that they were related to each other, and also to the protein S deficiency.