Background: Calciphylaxis is associated with end-stage renal failure or kidney transplant, although, cases of non-uremic calciphylaxis have been reported. It is a rare disorder, with high mortality, characterized by vascular calcification within cutaneous vessels; vascular calcification can occur in other organs, besides skin, like heart, lung, or pancreas, which is named visceral calciphylaxis.Objective: The purpose was to review current knowledge regarding diagnosis and therapeutic approach to cutaneous calciphylaxis.Methods: A literature review has been conducted associated to word \"calciphylaxis\".Results: The diagnosis is based mainly on clinical features and histologic findings. Therapeutic options are still controversial.Conclusions: Cutaneous calciphylaxis is a puzzling disease, with a challenging diagnosis, and a complex treatment, which requires a multidisciplinary team and expertise.

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