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(Circulation. 2001;103:2867.)
© 2001 American Heart Association, Inc.

Images in Cardiovascular Medicine

Rare Cause of Cardiomyopathy

E. B. Wu, MD; L. C. Fuller, MD; R. A. C. Hughes, MD; J. B. Chambers, MD

From the Cardiothoracic Centre (E.B.W., J.B.C.), Guy’s and St Thomas’ Hospitals; the Department of Dermatology, King’s College Hospital (L.C.F.); and the Department of Neuroimmunology, Guy’s, King’s, and St Thomas’ School of Medicine, London, UK.

Correspondence to Dr J.B. Chambers, Cardiothoracic Centre, 6th Floor, East Wing, St Thomas’ Hospital, Lambeth Palace Road, London SE1 7EH, UK. E-mail johnchambers{at}dial.pipex.com

A 62-year-old man with scleredema of Buschke was referred to the cardiology clinic because of an abnormal ECG. He had no cardiac symptoms, and the cardiovascular examination was unremarkable. The echocardiogram showed a dilated, nonhypertrophied left ventricle with no regional wall motion abnormalities and an estimated ejection fraction of 15%. The right ventricle was normal in size and systolic function. Coronary angiography showed significant proximal disease in the right coronary artery and distal circumflex artery. The left main stem and left anterior descending arteries were angiographically normal. Myocardial biopsies taken from the right ventricle showed histology typical of scleredema of Bushke, which has not previously been shown antemortem in the heart (Figure).

View larger version (112K): [in this window] [in a new window]   Figure 1. Top left, Dorsum of the back showing thickening of the skin as a result of scleredema of Buschke, a rare connective tissue disease characterized by mucin deposition in and around the collagen bundles of the dermis. Top right, ECG showing right bundle branch block with left anterior hemiblock. Bottom right, M-mode echocardiogram showing an end-diastolic dimension of 60 mm and an end-systolic dimension of 57 mm (fractional shortening, 5%). Bottom left, Histological section showing irregular myofiber hypertrophy with nuclear pleomorphism and lipofuscin deposits staining positive for acid mucopolysaccharide with alcian blue.

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