Leg ulcers in Klinefelter's syndrome--further evidence for an involvement of plasminogen activator inhibitor-1.
An abnormality in platelet aggregability or fibrinolysis, namely elevated activity of plasminogen activator inhibitor-1 (PAI-1), has been recently documented in patients suffering from Klinefelter's syndrome associated with leg ulceration without underlying venous insufficiency. To determine whether increased PAI-1 activity is a general feature of Klinefelter's syndrome, or more specifically associated with leg ulceration, we investigated PAI-1 influencing parameters and PAI-1 activity in two groups of patients: (i) Klinefelter patients suffering from leg ulceration (n = 7); and (ii) Klinefelter patients without leg ulceration (n = 6). On analysing PAI-1 influencing parameters such as age, body mass index, triglycerides, C-reactive protein, testosterone, smoking behaviour, the presence of diabetes mellitus, and arterial hypertension, respectively, we found no statistically significant differences between the two groups. However, PAI-1 activity in group 1 was highly significantly elevated compared with that in group two patients (P < 0.005). We conclude that (i) PAI-1 activity is not elevated in Klinefelter's syndrome in general; (ii) elevation of PAI-1 activity in patients suffering from Klinefelter's syndrome does not appear to be secondary to PAI-1 influencing parameters; and (iii) elevation of PAI-1 activity may play a crucial role in the pathogenesis of leg ulceration in Klinefelter's syndrome. Therefore, a therapy for leg ulceration in Klinefelter's syndrome that aims to return the elevated PAI-1 activity to normal should be explored.
Zollner
T M
TM
Department of Dermatology, University of Frankfurt Medical School, Germany.
Veraart
J C
JC
Wolter
M
M
Hesse
S
S
Villemur
B
B
Wenke
A
A
Werner
R J
RJ
Boehncke
W H
WH
Jost
S S
SS
Scharrer
I
I
Kaufmann
R
R
D001157:Arterial Occlusive Diseases / Q000150:complications / Q000378:metabolism; D015992:Body Mass Index; D048909:Diabetes Complications; D003920:Diabetes Mellitus / Q000378:metabolism; D005342:Fibrinolysis; D006801:Humans; D006973:Hypertension / Q000150:complications / Q000378:metabolism; D007713:Klinefelter Syndrome / Q000150:complications* / Q000378:metabolism; D007871:Leg Ulcer / Q000209:etiology* / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D017395:Plasminogen Activator Inhibitor 1 / Q000378:metabolism*; D012907:Smoking; D014280:Triglycerides / Q000378:metabolism
D017395:Plasminogen Activator Inhibitor 1; D014280:Triglycerides