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 prot ein S deficiency who presented with fever and chills, in whom idiopathic py lephlebitis was diagnosed. A year later, the patient was readmitted because of recurrent vomiting and weight loss. An exploratory laparotomy yielded d iagnosis of sclerosing peritonitis, which resolved after surgery. The short time interval between the processes suggests that they were related to eac h other, and also to the protein S deficiency.