Successful treatment with bosentan of non-digital skin ulcers in severe scleroderma

摘要

Recurrent digital ulcers are a manifestation of vascular disease in patients with systemic sclerosis leading to severe pain, impaired function and tissue loss. Treatment with the endothelin receptor antagonist, bosentan, has recently been shown to be of benefit in patients with severe Raynaud phenomenon and in the prevention and treatment of digital ulcers in systemic sclerosis. To our knowledge, there are no reports in the English literature on the use of bosentan on non-digital skin ulcers in patients with systemic sclerosis.A 62-year-old woman who had long-standing systemic sclerosis presented with a 2-month history of a large progressivepainful pretibial ulcer. Similar ulcerations in the past were treated with nifedipine, clopidogrel, antibiotics, local dressings including plastic gels, disinfection, vitamin cocktails, and courses of tramadol for pain. She also received iloprost in the past and underwent cervical sympathectomy for similar lesions. Laboratory evaluation showed haemoglobin of 10.5 g/dl (normocytic)and an erythrocyte sedimentation rate of 86. Testing for anti-centromere antibodies was positive; and testing for anti-cardiolipin antibodies was negative.