Calcinosis cutis associated with secondary hyperparathyroidism due to renal failure

A 28-year-old man with rejection of a renal transplant and end-stage liver disease was admitted for nephrectomy and a liver biopsy to determine the etiology of his liver disease. He was referred with a 4-month history of small, skin-colored papules that enlarged into painful sclerotic plaques. Physical examination revealed large violaceous, slightly hyperpigmented, firm, woody, inelastic plaques over the right axilla, left neck, popliteal fossa, and bilateral buttocks. No necrosis or ulceration was noted. Other physical findings included generalized icterus, ascites, muscle weakness and atrophy, and cachexia. Examination of skin biopsies from several locations revealed large areas in the dermis being replaced by histiocytic cells and large masses of calcium. The vasculature was not involved. The condition was diagnosed as metastatic calcinosis cutis. Elevated serum calcium and phosphorus levels were noted throughout his hospital course (Table 1). The calcium/ phosphate product (90.6 on admission) was high enough to produce an environment consistent with calcium deposition in soft tissue. Other significant laboratory values were: alkaline phosphatase 749 units per mL, direct bilirubin 12.2 mg per 100 mL, total bilirubin 17.2 mg per 100 mL, serum glutamate-oxaloacetic transaminase (SGOT) 273, serum glutamate-pyruvate transaminase (SGPT) 265, blood urea nitrogen (BUN) 210 mg per 100 mL, creatinine 10.3 mg per 100 mL, hemoglobin 6.4 g per L, and hematocrit 18. The parathyroid hormone level was elevated at 8.2 ng per mL (normal <.9). This finding was consistent with secondary hyperparathyroidism. The lesions progressed to form large, indurated, firm plaques with overlying erythematous skin involving a large portion of the skin surface. Increased pain with progressive involvement of the neck and extremities (particularly the knees and elbows) severely limited mobility. There was never a breakdown of overlying skin. The patient was first placed on aluminum hydroxide in an attempt to lower the serum phosphorus levels. He was dialyzed every other day with low calcium concentrations in the dialyzing fluid. After a minimal response, calcium acetate was added to help bind phosphorus. Parathyroidectomy was not performed because of the patient's unstable condition. The patient developed complications from anemia secondary to esophageal varices and bleeding from the transplanted kidney, sepsis from multiple resistant bacterial organisms, and left lower lobe pneumonia. The patient expired 1 month after admission. The transplanted kidney was removed 2 weeks before the patient's death. The histopathology revealed tubulo-interstitial fibrosis with a mild lymphoid cell infiltrate. No calcium deposition was noted in the kidney.