Cutaneous calciphylaxis treated by autologous keratinocytes graft and subtotal parathyroidectomy

BACKGROUND: Cutaneous calciphylaxis, seen most often in dialysis patients, is characterised by skin necrosis, and is a disabling and lifethreatening disease. Despite intensive topical treatment, recourse to parathyroidectomy is often necessary. We report the case of a female patient with skin necrosis due to calciphylaxis: pain and necrotic lesions were controlled by grafting of cultured autologous keratinocytes (Epibase). CASE REPORT: A 75-year-old woman with a 5-year history of dialysis-dependent chronic renal failure secondary to nephroangiosclerosis presented a very painful necrotic ulceration on her left leg. In spite of an autologous patch grafts, the lesions rapidly deteriorated. Laboratory data showed high levels of calcium, phosphate and parathyroid hormone and imaging suggested parathyroidal adenoma. Although cutaneous biopsy was not performed, the diagnosis of cutaneous necrosis due to calciphylaxis with tertiary hyperparathyroidism was established. Treatment consisted of a low-calcium and low-phosphorus diet with autologous keratinocytes grafts (Epibase). After the third application of keratinocytes, the pain disappeared, necrosis ceased and wound healing began. Subtotal parathyroidectomy was performed two months after the start of grafts. At three months, the patient was cured. DISCUSSION:. Calciphylaxis is an obstructive vascular disease secondary to calcification of the arterioles leading to ischemic tissue necrosis. Prompt diagnosis is essential since this disease is disabling and life-threatening due to sepsis and ischemic complications. In our case, autologous keratinocyte grafts allowed pain relief to be achieved within large expanses of cutaneous necrosis after correction of calcium and phosphorus levels, thereby allowing parathyroidectomy to be performed under optimal conditions.