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A painful skin lesion

Published:August 11, 2016DOI:https://doi.org/10.1016/j.ejim.2016.07.016

      1. Introduction

      A 64-year-old man with a history of arterial hypertension; diabetes mellitus; chronic kidney disease stage V; dilated, non-ischemic cardiomyopathy; status post-aortic valve replacement, was treated in our outpatient pre-dialysis clinic. He presented with volume overload and a necrotizing, non-healing skin ulcer. He had been treated with warfarin for several years due to the mechanical valve. The patient complained of severe leg pain and denied any trauma. He reported that the skin ulcer had developed from a small, erythematous plaque to a necrotizing lesion over several days. He was hospitalized in an Internal Medicine ward and began hemodialysis due to uremia and volume overload. During hospitalization the skin ulcer progressed. An ulcer developed in the other leg despite local treatment, including antibiotics (Fig. 1).
      Fig. 1
      Fig. 1Development of skin ulcers in both legs during hospitalization.
      What is the diagnosis?

      2. Diagnosis

      Calcific uremic arteriolopathy (calciphylaxis) is a rare, severe and often fatal skin condition associated predominantly, but not exclusively, with dialysis and chronic kidney disease [
      • Smith J.R.
      • Findlay M.D.
      • Geddes C.C.
      • Fox J.G.
      The role of sodium thiosulphate in the treatment of calciphylaxis.
      ].
      Calciphylaxis is characterized by painful, violaceous skin lesions, most commonly involving the lower limbs or trunk. The ulcerative lesions become infected and can lead to septicemia and death. One-year survival is about 30% [
      • Smith J.R.
      • Findlay M.D.
      • Geddes C.C.
      • Fox J.G.
      The role of sodium thiosulphate in the treatment of calciphylaxis.
      ,
      • Araya C.E.
      • Fennell R.S.
      • Neiberger R.E.
      • Dharnidharka V.R.
      Sodium thiosulfate treatment for calcific uremic arteriolopathy in children and young adults.
      ].
      This life-threatening condition represents small vessel vasculopathy characterized by intimal proliferation and medial wall calcification, which results in ischemia and necrosis of the skin and subcutaneous tissue [
      • Araya C.E.
      • Fennell R.S.
      • Neiberger R.E.
      • Dharnidharka V.R.
      Sodium thiosulfate treatment for calcific uremic arteriolopathy in children and young adults.
      ].
      Clinical suspicion should be aroused in CKD patients presenting with a painful skin lesion. Calciphylaxis can be very hard to diagnose in CKD and dialysis patients. The differential diagnosis includes warfarin-induced skin necrosis, peripheral vascular disease, vasculitis, cellulitis and atheroemboli. Clinical examination is the cornerstone of identifying calciphylaxis. Skin biopsy might be falsely negative and might increase the risk of infection and mortality.
      Risk factors for calciphylaxis include diabetes, obesity, hyperparathyroidism, hypercalcemia, hyperphosphatemia and medications such as warfarin, corticosteroids and calcium containing supplements [
      • Sprague S.M.
      Painful skin ulcers in a hemodialysis patient.
      ].
      The optimal treatment for calciphylaxis is not known. Treatment strategies include correction of underlying abnormalities in plasma calcium, phosphorus and PTH concentrations, aggressive wound care, adequate pain control, hyperbaric oxygen therapy and sodium thiosulfate (ST) [
      • Smith J.R.
      • Findlay M.D.
      • Geddes C.C.
      • Fox J.G.
      The role of sodium thiosulphate in the treatment of calciphylaxis.
      ].
      Due to a high index of suspicion our patient was managed using ST, with complete resolution of the lesions.

      Conflict of interests

      The authors state that they have no conflicts of interest.

      References

        • Smith J.R.
        • Findlay M.D.
        • Geddes C.C.
        • Fox J.G.
        The role of sodium thiosulphate in the treatment of calciphylaxis.
        Port J Nephrol Hypert. 2012; 26: 245-254
        • Araya C.E.
        • Fennell R.S.
        • Neiberger R.E.
        • Dharnidharka V.R.
        Sodium thiosulfate treatment for calcific uremic arteriolopathy in children and young adults.
        Clin J Am Soc Nephrol. 2006; 1: 1161-1166
        • Sprague S.M.
        Painful skin ulcers in a hemodialysis patient.
        Clin J Am Soc Nephrol. 2014; 9: 166-173