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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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