[Stomach perforation in generalized primary amyloidosis]

Even severe generalized amyloidoses are difficult to diagnose clinically. By way of example the case is reported of a 64-year-old patient hospitalized for severe abdominal pain who died within a few weeks of heart and circulatory failure. The unusual disease picture is dominated by three cardinal symptoms: large, hemorrhagic-bullous lesions of the skin, recurrent ventricular ulcers with perforation and local peritonitis, and progressive heart failure refractory to therapy. Pathologico-anatomically, amyloid deposits were found in all three organs and primary systemic amyloidosis was diagnosed. Stenotic deposits in the blood vessels and extensive involvement of the ventricular wall suggest that the ulcer is a consequence of the primary disease. The cases of amyloidosis found at autopsy at the Institute of Pathology, Zurich, over the last seven years are reviewed.