INCREASED PLASMA ACTIVITY OF PLASMINOGEN-ACTIVATOR INHIBITOR-1 (PAI-1) IN 2 PATIENTS WITH KLINEFELTERS-SYNDROME COMPLICATED BY LEG ULCERS

Klinefelter's syndrome is the most frequent major abnormality of sexua l differentiation in men with two or more X-chromosomes, and affects o ne in 500 males. The syndrome is characterized by eunuchoid body propo rtions, scanty facial and body hair, gynaecomastia, and small firm tes tes. Leg ulcers, especially in combination with hyperpigmentation, hav e been reported in association with Klinefelter's syndrome. Thromboemb olic processes are also frequently observed. The leg ulcers in patient s with Klinefelter's syndrome are usually attributed to venous insuffi ciency. We describe two patients with Klinefelter's syndrome associate d with recurrent ulcers and hyperpigmentation on both legs, in whom no venous or other underlying cause could be found. The patients were no t taking any drugs, in particular no supplemental androgen therapy. Bo th had normal plasma testosterone values. We detected increased activi ty of plasminogen activator inhibitor 1 (PAI-1), with only a partial d ecrease upon venous occlusion. A possible role for this inhibitor of f ibrinolysis in the pathogenesis of ulceration is discussed.