Multifocal Fibrosclerosis with Subcutaneous Involvement

Abstract

A 30-year-old woman developed progressive inferior and superior vena caval obstruction over a 5-month period. This was followed by evidence of hepatic dysfunction and, shortly before her death, by renal failure. The autopsy findings included mediastinal and retroperitoneal fibrosis with obstruction of both vena cavae and the renal, portal, and hepatic veins. The intrahepatic bile ducts were surrounded by scar tissue. The unique feature of this case was fibrosis of the abdominal subcutaneous adipose tissue, a finding previously unreported. The fibrosclerotic diseases are morphologic inflammations which clinically occasionally behave in an aggressive fashion simulating malignant neoplasms. In spite of considerable speculation concerning their pathogenesis, they remain idiopathic.