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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  • Published: 2018-12-04

    Issue: LATEST ONLINE (view)

    Section: Articles

    How to cite:
    Gomes, F., La Feria, P., Costa, C., & Santos, R. (2018). Non-Uremic Calciphylaxis: A Rare Diagnosis with Limited Therapeutic Strategies. European Journal of Case Reports in Internal Medicine, 2. https://doi.org/https://doi.org/10.12890/2018_000986