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Calciphylaxis is a syndrome of vascular calcification, thrombosis and skin necrosis. It is seen almost exclusively in patients with Stage 5 chronic kidney disease. It results in chronic non-healing wounds and is usually fatal. Calciphylaxis is a rare but serious disease.
Calciphylaxis is one type of extraskeletal calcification. Similar extraskeletal calcifications are observed in some patients with hypercalcaemic states, including patients with milk alkali syndrome, sarcoidosis, primary hyperparathyroidism, and hypervitaminosis D.
Calciphylaxis is characterised by
The cause is not known. It does not seem to be an immune type reaction. In other words, calciphylaxis is not a hypersensitivity reaction (i.e., allergic reaction) leading to sudden local calcification. Clearly, additional factors are involved in calciphylaxis. It is also known as calcific uraemic arteriolopathy; however, the disease is not limited to patients with kidney failure.
Calciphylaxis most commonly occurs in patients with end-stage renal disease who are on haemodialysis or who have recently received a renal transplant (= kidney transplant). Yet, calciphylaxis does not occur only in end-stage renal disease patients. It also has been reported in patients with breast cancer (treated with chemotherapy), liver cirrhosis (due to alcohol abuse), cholangiocarcinoma, Crohn’s disease, rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE) (including SLE patients with or without chronic renal disease).
There is no diagnostic test for calciphylaxis. The diagnosis is a clinical one. The characteristic lesions are the ischaemic skin lesions (usually with areas of skin necrosis). The necrotic skin lesions (ie, the dying or already dead skin areas) typically appear as violaceous (dark bluish purple) lesions and/or completely black leathery lesions. They can be extensive. The suspected diagnosis can be confirmed by a skin biopsy. It shows arterial calcification and occlusion in the absence of vasculitis. Sometimes the bone scintigraphy can show increased tracer accumulation in the soft tissues.[1]
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