[Leg ulcer and Klinefelter syndrome].
We report a case of 47 years old patient who was admitted to hospital because of bilateral leg ulcers for 6 years. Chromosome analysis revealed XXY karyotype, confirming the clinical diagnosis of Klinefelter's syndrome. Testosterone level was low and Plasminogen Activator Inhibitor (PAI-1) was elevated. The patient was given androgen therapy which resulted in a normalization of the PAI-1 activity. The frequency of leg ulcers in patients with Klinefelter syndrome is between 6 and 12% according to studies. Different causes would explain the tendency towards leg ulcers in Klinefelter's syndrome: conjunctive tissues abnormalities were revealed in some studies. A higher frequency of venous insufficiency is reported in patients with Klinefelter's syndrome, either due to the particular morphology (obesity, taller size) or due to an androgen deficiency. A few arterial dysplasias cases of arteries's legs were described in patients with leg ulcers and Klinefelter syndrome. Haemostasis disorders presented in this case and normalized after androgen therapy will contribute to the physiopathologic discussion.
Service de Chirurgie Vasculaire (Pr. Guidicelli), CHU, Grenoble.
B
B
Villemur
H
H
Truche
G
G
Pernod
M P
MP
De Angelis
J C
JC
Beani
S
S
Halimi
M
M
Collomb
A
A
Franco
H
H
Guidicelli
D000328:Adult; D000728:Androgens / Q000627:therapeutic use; D006801:Humans; D007621:Karyotyping; D007713:Klinefelter Syndrome / Q000188:drug therapy / Q000235:genetics*; D007871:Leg Ulcer / Q000235:genetics* / Q000473:pathology; D008297:Male
D000728:Androgens
Ulcère de jambe et syndrome de Klinefelter.