A 45-year-old female presented with a two-week history of painful skin lesions on both inner thighs. Her medical history was significant for alcoholic cirrhosis and hypothyroidism.
On physical exam, several tender violaceous plaques were present on the medial aspect of the left thigh ranging from 3 to 6 cm in largest dimension. On the right thigh, several firm, indurated contiguous plaques were appreciated along with one black necrotic lesion about 2 cm in diameter. No ulcerations, warmth or purulent drainage was noted. Laboratory investigation revealed an elevated phosphate but normal calcium, parathyroid hormone, calcium-phosphate product and renal function.
A skin biopsy was performed which showed calcium deposition in the tunica media layer of the arteries with surrounding fibrosis consistent with calciphylaxis. The patient received intravenous sodium thiosulfate treatment and Sevelamer. She was also started on an aggressive pain regimen. Bariatric oxygen treatment was also initiated as an outpatient.
Currently, there is very limited scientific data that can fully explain the enigma of calciphylaxis. Documented prevalence is highest amongst patients with chronic kidney disease (CKD) and end stage renal disease (ESRD), ranging from 1% to 4 %. Outside of this population of ESRD patients, calciphylaxis appears to be even more rare, with only four reported cases in patients with liver disease thus far.
One postulated theory is associated with the development of secondary hyperparathyroidism in patients with ESRD and CKD. Elevated parathyroid hormone causes bone resorption and accumulation of calcium and phosphate, resulting in an increased calcium-phosphate product. If the product is high enough (>70), calciphylaxis may occur. Hence, factors leading to disturbances in calcium and phosphorous homeostasis may contribute to the development of this syndrome.
Non-ESRD reported cases have been associated with primary hyperparathyroidism, cirrhosis, malignancy, immunosuppressive states and hypercoaguable states. Deficiency in protein C and protein S seem to play a role in the pathogenesis of calciphylaxis in these cases. Metha et al studied 12 patients with end-stage renal disease on dialysis and compared them to healthy volunteers. The study showed that patients with decreased levels of protein C or S are at increased risk of calciphylaxis, regardless of renal disease.
In conclusion, we have described a patient who developed calciphylaxis in association with alcoholic cirrhosis without any alteration of phosphocalcic metabolism. In the presence of liver disease, the ability to synthesize proteins necessary to activate and inhibit the coagulation cascade is diminished. Hence, low protein C and protein S can be a predisposing factor in cirrhotic patients that can lead to the development to calciphylaxis.
To cite this abstract:Baird P, Alam A, Tsomos E. Calciphylaxis Secondary to Alcoholic Cirrhosis. Abstract published at Hospital Medicine 2015, March 29-April 1, National Harbor, Md. Abstract 448. Journal of Hospital Medicine. 2015; 10 (suppl 2). https://www.shmabstracts.com/abstract/calciphylaxis-secondary-to-alcoholic-cirrhosis/. Accessed May 27, 2019.