Progressive systemic sclerosis (scleroderma), carcinoma breast and valvular heart disease: an unusual combination.

ABSTRACT

Systemic sclerosis is a multi system disorder characterised by fibrosis of skin and internal organs. There are reports of relation between cancer and polymyositis/dermatomyositis, but no overall association with systemic sclerosis. Reports of the coexistence of cancer and systemic sclerosis, however, emphasise a close temporal relation in their occurrence. Cardiac involvement in the form of myocardial fibrosis and pericarditis occurs frequently in systemic sclerosis, while valvular involvement has been reported only sporadically. We report a patient, admitted for adenocarcinoma of left breast who was found to have features of systemic sclerosis, pulmonary hypertension, gangrene of toes, and stenotic mitral valve disease. The possible mechanisms of the coincidence of the three disorders are discussed.