Calciphylaxis – A Rare Devastating Condition Associated with Chronic Kidney Disease

Abstract

Calciphylaxis, a rare, life threatening disease, usually observed in patients with chronic kidney disease and characterised by typical skin lesions -violaceous, reticulate areas of cutaneous necrosis particularly in the extremities, raised calcium phosphorous product, elevated parathyroid hormone level, histopathologically skin biopsies shows mural calcification affecting small arterioles and radiographic evidence of blood vessel and soft-tissue calcification.Calciphylaxis is a small-vessel vasculopathy, reported to occur in about 1-4% of haemodialysis patients. Disease is associated with a high mortality which ranges from 60-80% and relates to wound infection, sepsis and organ failure. Prolonged hyperphosphatemia and/or elevated calcium phosphorus products, Protein malnutrition, warfarin use and hypercoagulable states, such as protein C and/or protein S deficiency are associated with increased risk of the disease.Clinically, many dermatologic conditions resemble the skin lesions of Calciphylaxis but can be discern by careful clinical evaluation of patient and histopathological examination of skin biopsy. Different therapeutic agents have been reported for the treatment of this condition with variable results. Therapeutic goals are guided by controlling levels of parathyroid hormone, calcium, phosphate, and the calcium-phosphate product within the normal range. A high index of suspicion and an active multidisciplinary management approach, with rigorous attention to wound care and prevention of sepsis, are vital steps in the management of these patients.In this overview, we discuss the pathophysiology, clinical features, risk factors, and diagnosis and management issues relating to Calciphylaxis. This review is of interest to Medical specialist-specially to Dermatologist and Nephrologists for early identification of disease and therapeutic intervention.