Calcific uremic arteriolopathy (calciphylaxis) calls into question the validity of guidelines of diagnosis and treatment.

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. The purpose was to review current knowledge regarding diagnosis and therapeutic approach to cutaneous calciphylaxis. A literature review has been conducted associated to word "calciphylaxis". The diagnosis is based mainly on clinical features and histologic findings. Therapeutic options are still controversial. Cutaneous calciphylaxis is a puzzling disease, with a challenging diagnosis, and a complex treatment, which requires a multidisciplinary team and expertise.