FULMINANT METASTATIC CALCINOSIS WITH CUTANEOUS NECROSIS IN A CHILD WITH END-STAGE RENAL-DISEASE AND TERTIARY HYPERPARATHYROIDISM

Metastatic calcinosis is a common feature of chronic renal failure. It s first manifestations are bone demineralization and non-visceral and/ or visceral calcification with mostly mural deposits in arteries and a rterioles. It is initially characterized by hyperphosphataemia followe d by secondary or tertiary hyperparathyroidism. Cutaneous involvement is a rare complication, Histologically, the lesions show vascular calc ification with ischaemic skin necrosis, Extreme cases may produce calc inosis cutis (calciphylaxis), i.e. disseminated calcification of the s ubcutaneous tissue and dermis in the form of hard painful cutaneous no dules and plaques with subsequent ulceration. Metastatic calcinosis is a disease affecting adults, while the dystrophic or idiopathic type c an develop in children. We present the case of a 6-year-old boy with e nd-stage renal disease, attributed to congenital renal hypoplasia, and accompanied by secondary hyperparathyroidism. He developed fulminant tertiary hyperparathyroidism and metastatic calcinosis of the lungs, a s well as cutaneous necrosis of the buttocks and legs, subsequent to c alcification of arteries and arterioles. A maternal renal transplant f ailed to function. The serum parathormone, calcium and phosphate level s could not be controlled by maintenance dialysis, phosphate binders a nd calcitriol. Total parathyroidectomy without autotransplantation of parathyroid tissue rapidly returned the serum parathormone, calcium an d phosphate levels to normal. In addition, topical treatment using mer bromine solution and hydrocolloid dressings, healed the ulcers with si gnificant scar formation, within 2 . 5 months after parathyroidectomy. A renewed increase of the calcium x phosphate product, 2 months after parathyroidectomy, was attributed to mobilization of calcium compound s from the viscera, as confirmed by a chest X-ray.