Paget's disease of the breast with underlying carcinoma arising in systemic scleroderma

A case is presented of Paget's disease of the breast with underlying infiltrating carcinoma arising in a 35-year-old woman with systemic scleroderma. The tumor arose in an area of the skin affected by the systemic scleroderma 4 years after the onset of her systemic disease. The possibility of a causal relationship between these two processes is discussed and a brief review of the literature is presented.