Endocrine Abstracts

JOINT842

Introduction: Leg ulcers are chronic cutaneous defects with no spontaneous cicatrization. Management is often multidisciplinary, involving plastic surgeons, dermatologists and vascular surgeons. The endocrinologist will be required in cases of diabetes or associated complicating endocrinopathy. We report through this observation a patient with recurrent bilateral venous leg ulcers, in whom Klinefelter’s syndrome was confirmed.

Observation: 49-year-old patient, followed in our training for a klinefelter syndrome. Having a history of profound venous thrombosis under a venous contension. A recurrence of venous ulcer was the main reason for this hospitalisation. Venous Doppler revealed bilateral ostial incontinence and multiplevaricose veins with incontinent perforators. Management was medical, with compression between 20 and 30 mmHg. Prevention includedmaintenance of effective contention with anticoagulation since surgery was not an option. The evolution was favorable after equilibration of diabetes, testosterone administration andoffloading mechanical tissue stress. We discuss through this case the physiopathology of lower extremity ulcers in Klinefelter’ssyndrom.

Discussion and Conclusion: Klinefelter’s syndrome, first described in 1942 by Harry F. Klinefelter, is the most commoncongenital abnormality causing primary hypogonadism, occurring in 1 in 500 to 1 in 1000 livebirths. In a series of Klinefelter’s patients observed for up to a 20-year period, the prevalence ofleg ulcers was 6–13%. The etiology of lower limb ulcers in these patients is multifactorial, with chronic venousinsufficiency, obesity, arteriopathy and, in particular, reduced fibrinolysis due to high levels of PAI-1, which may explain the thrombosis of cutaneous microvessels or venous thrombosis leading topost-phlebitic disease, as occurred in our patient. On the other hand, there is a loss of endothelial inflammatory control due to the decrease oftestosterone levels, as confirmed by the improvement under androgen therapy. This case illustrates an under- recognized complication of Klinefelter’s syndrome. Thepathogenesis of ulcers in this endocrinopathy is unclear, but associations with abnormalities of fibrinolysis and prothrombotic states are reported.