Pitfall of diagnostic medicine: an uncommon cause of legs ulcers

Eva Larrañaga, Jean-Baptiste Nicolas, Julian Muguerza Published in the journal : April 2020 Category : General Internal Medicine

Summary :

Calciphylaxis is an uncommon and complex syndrome characterized by the occlusion of micro-vessels within the subcutaneous tissue, resulting in painful ischemic skin ulcers. Its prognosis is poor, and the disease is associated with a high mortality rate. This rare condition usually affects patients with chronic kidney disease, but it may also occur in patients with normal kidney function. The pathogenesis of calciphylaxis is unlikely to be linked to vascular calcifications, which are frequently seen in the general population. Therapeutic guidelines are still lacking, whereas recent studies have improved the understanding of the condition’s physiopathology, enabling us to expect encouraging therapeutic approaches. Through a clinical case, this paper highlights the condition’s diagnostic difficulties and discusses its physiopathology, as well as treatment procedures.

Key Words

Calciphylaxis, calcific non-uremic arteriolopathy, risk factors, sodium thiosulfate

What is already known about the topic?

alciphylaxis is an uncommon condition with poor prognosis, commonly under-diagnosed, as it is poorly known. Once suspected, skin biopsy is required for diagnostic confirmation. Therapeutic guidelines are still lacking. A multi-disciplinary approach is recommended, including control of associated risk factors, analgesics, wound management, as well as use of pharmacological agents.

What does this article bring up for us?

This article summarizes the clinical characteristics of calciphylaxis and its risk factors, reviews the condition’s physiopathology and explains its therapeutic approaches. This article seeks to raise awareness about this rare and challenging condition, as clinical suspicion is decisive for early diagnosis and optimized treatment.