[Systemic Buschke's scleredema with cardiomyopathy, monoclonal IgG kappa gammopathy and amyloidosis. Case report with autopsy]

Abstract

A 73 year old retired truck driver and blacksmith was studied in June 1996 for thoracic pain and was diagnosed as acute pericarditis which responded 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 component, 1.31 g/oo. TSH and tetraiodotironine were normal; ESR 16 mm in the first hour. As he did not respond to treatment he was referred to our hospital in March 1997. On physical examination the most relevant findings were a non-pitting edema of the abdomen and lower limbs, sparing the feet. An echocardiogram was consistent with an infiltrative cardiomyopathy. Soon after his hospitalization his condition worsened suddenly with severe bradicardia (28/minute) due to a junctional rhythm and righ bundle branch block. He suffered a cardiac arrest and died. The autopsy findings favoured the diagnosis of systemic scleredema adultorum of Buschke. Amyloid deposits were also found although not abundant, with a similar distribution except in the skin. In this article the clinical and autopsy findings are presented in a patient showing coexistence of systemic Buschke's scleredema with an infiltrative cardiomyopathy, IgG Kappa gammopathy and amyloidosis.