A man with Klinefelter's syndrome (KS) may develop recurrent leg and foot cutaneous ulcers.
Clinical features:
(1) male with Klinefelter's syndrome
(2) chronic, recurrent ulcers over the legs and feet
(3) low serum concentration of testosterone
(4) high serum concentrations of plasminogen activator inhibitor-1 (PAI-1), which is associated with venous thromboemboli secondary to impaired fibrinolysis
The differential diagnosis includes other causes of hypercoagulability, vasculitis, venous insufficiency and cryoglobulinemia.
Therapy with testosterone normalizes (reduces) the PAI-1 levels and results in resolution of the skin ulcerations.
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