Calciphylaxis associated with cholangiocarcinoma treated with low-molecular-weight heparin and vitamin K

Calciphylaxis is a rare disorder of small-vessel calcification and cutaneou s infarction associated with chronic renal failure. Rare cases of calciphyl axis not associated with chronic renal failure have been reported with brea st cancer, hyperparathyroidism, and alcoholic cirrhosis, To our knowledge, we report the first case of calciphylaxis without chronic renal failure ass ociated with cholangiocarcinoma and the first attempt to treat calciphylaxi s with vitamin K, A 56-year-old woman presented with necrotic leg ulceratio n. She was treated initially with low-molecular-weight heparin, with no eff ect. A coagulation work-up showed vitamin K deficiency. During vitamin K th erapy, the patient had fulminant progression of the calciphylaxis. She died , and an autopsy showed metastatic cholangiocarcinoma. Thrombosis and prote in C deficiency have been implicated in the pathophysiology of calciphylaxi s, Functional protein C deficiency may be one of several factors contributi ng to the development of calciphylaxis. Vitamin K therapy was ineffective i n our patient and may have been detrimental.