SYSTEMIC BUSCHKES SCLEREDEMA WITH CARDIOMYOPATHY, MONOCLONAL IGG KAPPA GAMMOPATHY AND AMYLOIDOSIS - CASE-REPORT WITH AUTOPSY

A 73 year old retired truck driver and blacksmith was studied in June 1996 for thoracic pain and was diagnosed as acute pericarditis which r esponded well to steroid treatment. In January 1997, he noted swelling of the abdominal skin, genitalia and limbs, sparing the feet. He was euthyroid, did not have evidence of diabetes or a Raynaud's phenomenon . His proteinogram showed an IgG-Kappa monoclonal paraprotein M compon ent, 1.31 g/oo. TSH and tetraiodotironine were normal; ESR 16 mm in th e first hour. As he did not respond to treatment he was referred to ou r hospital in March 1997. On physical examination the most relevant fi ndings were a non-pitting edema of the abdomen and lower limbs, sparin g the feet. An echocardiogram was consistent with an infiltrative card iomyopathy. Soon after his hospitalization his condition worsened sudd enly with severe bradicardia (28/minute) due to a junctional rhythm an d right bundle branch block. He suffered a cardiac arrest and died. Th e autopsy findings favoured the diagnosis of systemic scleredema adult orum of Buschke. Amyloid deposits were also found although not abundan t, with a similar distribution except in the skin. In this article the clinical and autopsy findings are presented in a patient showing coex istence of systemic Buschke's scleredema with an infiltrative cardiomy opathy, IgG Kappa gammopathy and amyloidosis.