Published online Jan 27, 2019. doi: 10.4254/wjh.v11.i1.127
Peer-review started: September 4, 2018
First decision: October 15, 2018
Revised: December 2, 2018
Accepted: January 3, 2019
Article in press: January 4, 2019
Published online: January 27, 2019
Processing time: 145 Days and 11.1 Hours
Calciphylaxis is a form of vascular calcification more commonly associated with renal disease. While the exact mechanism of calciphylaxis is poorly understood, most cases are due to end stage kidney disease. However, it can also be found in patients without kidney disease and in such cases is termed non-uremic calciphylaxis for which have multiple proposed etiologies.
We describe a case of a thirty-year-old morbidly obese Caucasian female who had a positive history of alcoholic hepatitis and presented with painful calciphylaxis wounds of the abdomen, hips, and thighs. The hypercoagulability panel showed low levels of Protein C and normal Protein S, low Antithrombin III and positive lupus anticoagulant and negative anticardiolipin. Wound biopsy confirmed the diagnosis of non-uremic calciphylaxis in the setting of alcoholic liver disease. The calciphylaxis wounds did not improve when Sodium Thiosulfate was used alone. The patient underwent a series of bedside and surgical debridement. Broad spectrum antibiotics were also used for secondary wound bacterial infections. The patient passed away shortly after due to sepsis and multiorgan failure.
Non-uremic Calciphylaxis can occur in the setting of alcoholic liver disease. The treatment of choice is still unknown.
Core tip: In this case report, we present a patient with alcoholic liver disease and low levels of Protein C who developed calciphylaxis and died shortly after due to complications. The pathogenesis is not completely understood but the disruption of calcium-phosphate-byproduct has been implicated to play a role in the disease process. Liver dysfunction can lead to low levels of coagulation inhibitors specifically Protein C and Protein S. The aim of the medical treatment is to lower the calcium-phosphate-byproduct and decrease the vascular calcification. The use of surgical wound debridement is less established.