Scleroderma was first described centuries ago. The name literally means "hard skin" even though the disease may progress to involve numerous organs and sometimes rapidly leading to the patients' demise. It is classified as localized (morphea, generalized morphea, linear scleroderma) and systemic (acrosclerosis and diffuse sclerodema).

The incidence of scleroderma and malignant conditions has been seldom described and sporadically (unlike the well-recognized relation between malignancy and dermatomyositis).

Several case-reports, as well as later controlled larger studies, reported a significant coincidence between scleroderma and certain types of cancer, with a frequency rate between 3-7% [1].

The most frequent malignancy reportedly associated with PSS is lung cancer.

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