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Increased plasma activity of plasminogen activator inhibitor 1 (PAI‐1) in two patients with Klinefelter's syndrome complicated by leg ulcers
Author(s) -
VERAART J.C.J.M.,
HAMULYAK K.,
NEUMANN H.A.M.,
ENGELEN J.
Publication year - 1994
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.1994.tb13112.x
Subject(s) - klinefelter syndrome , medicine , plasminogen activator , fibrinolysis , hyperpigmentation , gynecomastia , androgen deficiency , endocrinology , dermatology , skin hyperpigmentation , gastroenterology , androgen , hormone
Summary Klinefelter's syndrome is the most frequent major abnormality of sexual differentiation in men with two or more X‐chromosomes, and effects one in 500 males. The syndrome is characterized by eunuchoaid body proportions, scanty facial and body hair, gynaecomastia, and small firm testes. Leg ulcers, especially in combination with hyperpigmentation, have been reported in association with Klinefelter's syndrome. Thromboembolic processes are also frequently observed. The leg ulcers in patients with Klinefelter's syndrome are usually attributed to venous insufficiency. We describe two patients with Klinefelter's syndrome associated with recurrent ulcers and hyperpigmentation on both legs, in whom no venous or other underlying cause could he Found. The patients were not taking any drugs, in particular no supplemental androgen therapy. Both had normal plasma testosterone values. We detected increased activity of plasminogen activator inhibitor 1 (PAI‐1). with only a partial decrease upon venous occlusion. A possible role for this inhibitor of fibrinolysis in the pathogenesis of ulceration is discussed.