Non-Uremic Calciphylaxis: A Rare Diagnosis with Limited Therapeutic Strategies
  • Felisbela Miguel Gomes
    Sociedade Portuguesa de Medicina Interna
  • Pedro La Feria
    Unidade Funcional de Medicina 2.1, Hospital de Santo António dos Capuchos, Centro Hospitalar de Lisboa Central, Lisbon, Portugal
  • Catarina Costa
    Unidade Funcional de Medicina 2.1, Hospital de Santo António dos Capuchos, Centro Hospitalar de Lisboa Central, Lisbon, Portugal
  • Rita Santos
    Unidade Funcional de Medicina 2.1, Hospital de Santo António dos Capuchos, Centro Hospitalar de Lisboa Central, Lisbon, Portugal

Keywords

Calciphylaxis, leg ulcer, vascular calcification, sepsis

Abstract

Calciphylaxis is a rare condition characterized by the emergence of non-healing skin ulcers secondary to arterial calcification and thrombosis, typically diagnosed in patients with end-stage kidney disease (ESKD). When it develops in patients without ESKD, it is called non-uremic calciphylaxis (NUC). The latter is an even rarer diagnosis with an uncertain pathophysiology and a high mortality rate (52%), mainly due to sepsis (50%). Cutaneous biopsy is diagnostic. Therapeutic measures recommended for NUC are limited to wound debridement, analgesia, and control of infection and risk factors. Other therapeutic options exist but with a low level of evidence. We present the case of a 78-year-old woman with NUC in her lower limbs who died of sepsis. NUC is a therapeutic challenge lacking efficient strategies.

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References

  • Nigwekar SU, Kroshinsky D, Nazarian RM, Goverman J, Malhotra R, Jackson VA, et al. Calciphylaxis: risk factors, diagnosis, and treatment. Am J Kidney Dis 2015;66:133–146.

  • Nigwekar SU, Wolf M, Sterns RH, Hix JK. Calciphylaxis from nonuremic causes: a systematic review. Clin J Am Soc Nephrol 2008;3:1139–1143.

  • Wollina U. Update on cutaneous calciphylaxis. Indian J Dermatol 2013;58:87–92.

  • Lee JL, Naguwa SM, Cheema G, Gershwin ME. Recognizing calcific uremic arteriolopathy in autoimmune disease: an emerging mimicker of vasculitis. Autoimmun Rev 2008;7:638–643.

  • Marques SA, Kakuda AC, Mendaçolli TJ, Abbade LP, Marques ME. Calciphylaxis: a rare but potentially fatal event of chronic kidney disease. Case report. An Bras Dermatol 2013;88(6 Suppl 1):44–47.

  • Weenig RH. Pathogenesis of calciphylaxis: Hans Selye to nuclear factor kappa-B. J Am Acad Dermatol 2008;58:458–471.

  • Schlieper G, Brandenburg V, Ketteler M, Floege J. Sodium thiosulfate in the treatment of calcific uremic arteriolopathy. Nat Rev Nephrol 2009;5:539–543.

  • Vedvyas C, Winterfield LS, Vleugels RA. Calciphylaxis: a systematic review of existing and emerging therapies. J Am Acad Dermatol 2012;67:e253–260.

  • ISRCTN registry. ISRCTN73380053: A clinical trial with sodium thiosulfate for the treatment of calciphylaxis. Available from http://www.isrctn.com/ (accessed 13 Nov 2015).

  • Arenas MD, Gil MT, Gutiérrez MD, Malek T, Moledous A, Salinas A, Alvarez-Ude F. Management of calcific uremic arteriolopathy (calciphylaxis) with a combination of treatments, including hyperbaric oxygen therapy. Clin Nephrol 2008;70:261–264.

  • Chan MR, Ghandour F, Murali NS, Washburn M, Astor BC. Pilot study of the effect of lanthanum carbonate (Fosrenol®) in patients with calciphylaxis: a Wisconsin Network for Health Research (WiNHR) study. J Nephrol Ther 2014;4:1000162.

  • Nigwekar SU, Bhan I, Turchin A, Skentzos SC, Hajhosseiny R, Steele D, Nazarian RM, et al. Statin use and calcific uremic arteriolopathy: a matched case-control study. Am J Nephrol 2013;37:325–332.

  • Ross EA. Evolution of treatment strategies for calciphylaxis. Am J Nephrol 2011;34:460–467.

  • Salmhofer H, Franzen M, Hitzl W, Koller J, Kreymann B, Fend F, et al. Multi-modal treatment of calciphylaxis with sodium-thiosulfate, cinacalcet and sevelamer including long-term data. Kidney Blood Press Res 2013;37:346–359.

  • el-Azhary RA, Arthur AK, Davis MD, McEvoy MT, Gibson LE, Weaver AL, et al. Retrospective analysis of tissue plasminogen activator as an adjuvant treatment for calciphylaxis. JAMA Dermatol 2013;149:63–67.

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    Published: 2018-12-04
    Issue: Vol 5 No 12 (view)

    How to cite:
    1.
    Gomes FM, La Feria P, Costa C, Santos R. Non-Uremic Calciphylaxis: A Rare Diagnosis with Limited Therapeutic Strategies. EJCRIM 2018;5 doi:10.12890/2018_000986.

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