Article Text
Abstract
Background Calciphylaxis is a rare and life-threatening condition of progressive cutaneous necrosis secondary to small and medium-sized vessel calcification seen almost exclusively in patients with end-stage renal disease and hyperparathyroidism.
Objectives We report a case with calciphylaxis due to long-term steroid using in a patient with rheumatoid arthritis (RA).
Methods Case report.
Results A 43-year-old man admitted with a history of progressive left leg pain and ulcerations. Several hemoragic bullae were present in these areas as well, and the patient offered that the ulcers began as blisters that then brokedown. His past medical history included RA and he had been using steroid in doses of 5–40 mg/day according to his complaints for 20 years when he was admitted to our hospital. His examination revealed destructive and erosive arthritis affecting his bilateral wrists, MCP, and PIF joints, and ischaemic necrosis in the distal parts of the 2nd. and 3th. digits of the left hand. Laboratory results were as follows: erythrocyte sedimentation rate 77 mm/h, urea 9 mg/dl, creatinine 0.8 mg/dl, calcium 7 mg/dl, phosphate 3.7 mg/dl, albumin 2 gr/dl, parathyroid hormone 43 pg/ml (n = 12–72), protein S 38% (n = 7–115%). Roentgenogram of the lower aspect of the leg showed calcified larger vessels and subcutaneous vessels. His lesions progressed and required above the left knee and left 2nd., 3th. digits amputations. Pathologic examination showed extensive arterial wall calcification. Now he has been under the treatment of sulfasalazine and he has remained asymptomatic.
Conclusion Calciphylaxis may appear without renal failure and hyperparathyroidism. Long-term steroid using in RA patients may lead to calciphylaxis. Acquired protein S deficiency may contribute to this process.