Klinefelter's syndrome associated with progressive systemic sclerosis: report of a case and review of the literature.

A case of Klinefelter's syndrome associated with progressive systemic sclerosis (PSS) is described. The patient had been infertile for 20 years after marriage and was diagnosed as PSS with Klinefelter's syndrome at the age of 43. In reviewing the literature, we observed that five cases with PSS within the range of 41 to 61 years of age, and seventeen cases with systemic lupus erythematosus (SLE), but only few cases with other connective tissue diseases were reported in association with Klinefelter's syndrome. Possible involvement of male hypogonadism in the pathogenesis of PSS was suggested.