Progressive systemic sclerosis (PSS) is a multi-organ disease of protean manifestations. Among its many effects, gastrointestinal involvement is common, and liver dysfunction has been seen on occasion. On the other hand, gastrointestinal hemorrhage is rare, and jaundice unequivocally due to PSS has not been described.
The present report concerns a patient with severe PSS for over eight years, whose terminal admission was for obstructive jaundice and gastrointestinal bleeding. At autopsy, both these clinical features were found to be attributable to the arterial lesions of PSS.
A 23-year-old Negro man was first seen at age 15 for a draining leg ulcer at the site of a burn treated by repeated skin grafting attempts. Dyspnea on exertion, joint aches, and stiffness, and tightness of the skin of the hands were also present at that time, and a skin biopsy was consistent with PSS. During the next eight years, until his