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

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 POINTSCalciphylaxis 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.

Nonuremiccalciphylaxis - A Systematic Review

“…We identified 53 cases of NUC [5]- [6][7]which included 46 (86.79 %) individual case reports and 7 (13.20 %) case series Fig. 1.…”

“…Most of the studies reported multiple conditions 18 (33.96 %) [8,5,9,10,11,[12][13][14]6,15,16,17,1819,20,21,22], [7]; define herein as patients suffering from more than two morbidities were assembled in multiple conditions. Other morbidities among the included studies were warfarin induced (7.54 %) [23,24,25 1).…”

“…It's more common in those with end-stage renal illness (ESRD). Calciphylaxis is a condition that causes painful skin lesions that eventually turn into ulcers [3][4][5][6][7][8]. The calcification of small and medium-sized dermal and subcutaneous veins, intimal hyperplasia, and thrombosis distinguishes this rare disease histologically.…”

<p>Management of patients with calciphylaxis: current perspectives</p>

“…Efficient therapeutic procedures and evidence-based recommendations are needed especially for non-uremic CP, since therapeutic strategies can so far only be transferred from CUA treatment without a pathophysiological correlation. 27 Concepts of a modern wound management should be followed with special emphasis on debridement and prevention or early treatment of wound infections which are involved in the high mortality of CP patients due to sepsis. Non-adhesive modern wound dressings or products with silicone layer can be applied to the wound to enable an atraumatic dressing change and reduce pain.…”

“…As the presented therapeutic strategies mainly derive from those for uremic CP, more efficient therapeutic measures and evidence-based recommendations are needed for non-uremic cases. 27 A clear diagnostic classification as CP is mandatory.…”