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            Calciphylaxis is a rare and progressive cutaneous necrosis secondary to small and medium sized vessel wall calcification seen almost exclusively in patients with endstage renal disease and hyperparathyroidism. Calciphylaxis was first described by Selye in 1962. There is a slight preponderance in women. The pathogenesis of calciphylaxis is not well understood. Several factors such as end stage renal disease, hyperparathyroidism, hyperphosphatemia, hypercalcemia, a high calcium phosphate product, nephrectomy, use of steroids, albumin infusions, iron dextran complex, calcium heparinate, vitamin D are all implicated in its genesis.

     Vascular calcification is common in uraemia occurring in upto 43 % of patients undergoing dialysis whereas calciphylaxis is rare.  It is likely that various factors may  interact in the clinical setting to initiate the cascade of events responsible for calciphylaxis. However the exact sequence in any individual patient is not known and difficult to implicate. Recently hyper-coagulability due to functional protein C deficiency has been suggested to play a role in the development of this condition.

Clinically calciphylaxis often begins as painful mottling of the skin resembling livedo reticularis. The distal extremities and legs are commonly affected and less commonly the thighs, abdomen, gluteal region and breasts. Acral gangrene, autoamputation of fingers, penile calcification have been reported to occur in calciphylaxis. In addition cerebral, myocardial, splenic and intestinal infarction have also been reported.

    Histological findings in calciphylaxis include the presence of calcification within small and medium blood vessels in the dermis and subcutis, intraluminal debris or fibrin thrombi may be seen in some vessels, fat necrosis has also been reported as a result of calciphylaxis. Sometimes serial sections may be required, as the number of calcified capillaries may be small.

     Treatment of calciphylaxis is disappointing, as no effective treatment is available. Parathyroidectomy helps some patients in early stages. Steroids are controversial and most reports contraindicate its usage;however  a short course of steroids for 10 days along with 3 months of cimetidine was  found effective in one isolated  report.

 
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