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Published on: November 7, 2017
Non-Uremic Calciphylaxis: A Rare Diagnosis with Limited Therapeutic Strategies
Felisbela Gomes1, Pedro La Feria1, Catarina Costa1
1Unidade Funcional de Medicina 2.1, Hospital de Santo António dos Capuchos, Centro Hospitalar de Lisboa Central, Lisbon, Portugal.
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.
Learning Points:
Calciphylaxis in the absence of end-stage kidney disease is called non-uremic calciphylaxis (NUC).This disease is a diagnostic and therapeutic challenge.As therapeutic strategies for NUC mainly derive from those for uremic calciphylaxis, more efficient therapeutic measures and evidence-based recommendations are needed.
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