Calciphylaxis in the Setting of Alcoholic Cirrhosis

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Calciphylaxis can be a severe life threatening dermatologic disease that is a known complication of end stage renal disease. However, non uremic etiologies can cause calciphylaxis. We report a rare case of a forty year old female with history of alcoholic cirrhosis without any evidence of renal dysfunction who presents with calciphylaxis. A 40 year old female with a history of cirrhosis secondary to progression of alcoholic hepatitis presented to the hospital complaining of painful non- healing but worsening ulcerations on her thighs. For the past several months, the patient has refrained from any type of alcohol abuse, which was represented by marked improvement in liver chemistries. On physical exam, the patient presented with no clinical characteristics of decompensated cirrhosis. The lower extremities were remarkable for a blotchy erythematous pattern in the upper thighs resembling levido reticularis. Extensive areas of eschar and skin necrosis were present and the lesions appeared indurated and were extremely tender to touch. The skin lesion on the thigh was biopsied and it showed ulceration and subcutaneous tissue with fat necrosis. Multiple medium sized vessels in the fat demonstrated prominent concentric calcification of the vascular wall, the characteristic pathological findings consistent with calciphylaxis. Our patient's case is remarkable because she did not have any history of chronic kidney disease and did not present with any transient renal dysfunction. The patient has no history of hyperparathyroidism, calcium or phosphorous related disorders. In alcohol induced liver disease related calciphylaxis, few case reports in the literature have shown that deficiencies in protein C and protein S levels result in vascular injury. Liver disease can lead to low synthesis of coagulation factors and other proteins that can lead to susceptibility to injury. All therapies have been directed towards addressing the elevated calcium and phosphorous levels. In calciphylaxis secondary to non-uremic etiologies, no affective treatment is available. Supportive treatments include wound care, pain control, and empiric antibiotics, a regimen that our patient received. No literature is available at this time to determine if a liver transplant in cirrhotic patients leads to complete resolution of the dermatologic disease. At this time, further investigative studies need to be done to properly understand the relationship between liver disease and calciphyalxis.Figure 1Figure 2Figure 3