Non-Uremic Calciphylaxis: A Rare Diagnosis with Limited Therapeutic Strategies

Authors

• 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

felisbelamiguelgomes@gmail.com

Keywords

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.