Ischemic digital ulcerations complicate 15% to 25% of cases of systemic sclerosis and can be difficult to treat with the usual vasodilators. Herein, we describe a patient with systemic sclerosis manifesting as digital ulcers refractory to conventional treatments who benefited from the new endothelin receptor antagonist bosentan (Tracleer; Actelion Pharmaceuticals Ltd, Allschwil, Switzerland).
A 39-old-woman was seen with a 2-month history of very painful digital ulcerations on both hands and Raynaud phenomenon for 10 years. On physical examination, a mild sclerodactyly was noted without telangiectases (Figure 1). Arteriography showed bilateral distal arteriopathy with occlusion of the left ulnar artery and other digital arteries. Test results for antinuclear antibodies were positive at 1/2000 without detectable antigen specificity.
Chamaillard M, Heliot-Hosten I, Constans J, Taïeb A. Bosentan as a Rescue Therapy in Scleroderma Refractory Digital Ulcers. Arch Dermatol. 2007;143(1):115-126. doi:10.1001/archderm.143.1.125