This article describes the classification and aetiology of systemic scleroderma and reviews the management of patients with this unusual rheumatic condition

The word scleroderma derives from the Greek words 'sclero' meaning hard and derma' meaning skin. It is an uncommon disease, with an incidence of 20 new cases per million people per year (Madsger 1992). It is characterised by an over-production of collagen, a major protein of connective tissue found in the skin, blood vessels, joints and internal organs including the kidneys, lungs and bowels.