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A 71-year-old woman received a diagnosis of systemic sclerosis more than 30 years ago when she presented with Raynaud's phenomenon and cutaneous sclerosis. At that time she did not require long-term therapy. She now presents with fever and venous ulceration of both legs. On physical examination it was discovered that the tips of all 10 fingers and some toes were missing. She reported no history of severe trauma or surgery to her fingers or toes. A test for antinuclear antibodies was positive at 1:1280, and though unusual, there were detectable levels of both anti-Scl70 antibodies and anticentromere antibodies; the presence of either would have confirmed the diagnosis of systemic sclerosis. There was no evidence of involvement of the kidneys or lungs. A radiograph of the left hand shows marked acral osteolysis, which is also present in the right hand. The fever and leg ulcerations improved with antimicrobial therapy and local care.
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