Abstract

We present a rare case of non-uremic calciphylaxis occurring in the setting of alcoholic liver cirrhosis without evidence of renal dysfunction.  A 59-year-old female with history of ascites and alcoholic cirrhosis presented with painful sores on back of her legs since, 3 months. Physical examination showed bilaterally distributed retiform purpura with ulceration and violaceous papules on distal calf. Clinical differential diagnosis included thrombotic vasculopathy, polyarteritis nodosa, antiphospholipid antibody syndrome, and pyoderma gangrenosum. Punch biopsy from the left calf showed evidence of ischemic tissue necrosis with reactive angiomatosis, dermal sclerosis, hemorrhage and fat necrosis.  There was prominent concentric calcification of the vascular walls of medium-sized vessels in the subcutis, consistent with calciphylaxis. Patient’s renal function and serum levels of calcium, phosphorous, parathormone, proteins C and S, and lupus anticoagulant were normal. Non-uremic causes of calciphylaxis reported in literature include alcoholic liver disease, protein C/S deficiency, vitamin D deficiency, malignancy, hyperthyroidism, diabetes, and Crohn’ s disease. Such cases are rare, but life-threatening dermatological conditions which are underrepresented in literature. Non-uremic cases of calciphylaxis tend to have a poor prognosis, with one literature resource citing a mortality rate of 52% with most deaths occurring between two weeks and one year after diagnosis. Clinical presentation of retiform purpura indicating ischemic tissue damage in patients with above mentioned conditions, even in the absence of renal dysfunction, should prompt evaluation for calciphylaxis.  Early recognition of calciphylaxis in non-uremic settings is essential for prompt treatment and patient outcome.