Background: Calciphylaxis is a rare thrombotic vasculopathy characterized by high morbidity and mortality. There is a paucity of studies examining longitudinal outcomes. Objective: To assess mortality, days spent in the hospital, and amputations in patients with calciphylaxis. Methods: A retrospective medical record review was conducted in 145 patients diagnosed with calciphylaxis at an urban tertiary care hospital from January 2006 to December 2018. Results: Six-month mortality was 37.2%, and 1-year mortality was 44.1%. Patients with nephrogenic calciphylaxis had worse survival than those with nonnephrogenic calciphylaxis (P = .007). This difference in survival disappeared when limiting mortality to deaths due to calciphylaxis. Age (P = .003) and end-stage renal disease (P = .01) were risk factors associated with 1-year mortality. Diabetes mellitus was associated with greater total hospitalization days (coefficient, 1.1; 95% confidence interval, 1.01-1.4); bedside debridement was associated with fewer hospitalization days (coefficient, 0.8; 95% confidence interval, 0.7-0.9). Amputations were not associated with any of the examined risk factors. The use of warfarin followed by a transition to nonwarfarin anticoagulation was associated with decreased hazard of death (P = .01). Limitations: Retrospective nature. Conclusions: Calciphylaxis remains a complex, heterogeneous disease. Mortality is lower in patients with nonnephrogenic disease. These findings may be incorporated during discussions regarding the goals of care to facilitate informed shared decision making.

Calciphylaxis is a rare life-threatening disorder characterized by skin necrosis due to calcium deposition within small- and medium-sized vessels and subsequent thrombosis leading to tissue ischemia.1,2 Though the exact pathogenesis of calciphylaxis is poorly understood, risk factors such as chronic kidney disease can promote calcium deposition and vascular calcification contributing to the disease process.3 This condition presents with poor healing, painful skin lesions, which initially may be confused with mimickers, such as cellulitis; peripheral vascular disease, warfarin necrosis, or vasculitis.4 While calciphylaxis is considered a rare disease predominantly affecting patients with chronic kidney disease on renal replacement therapy, there has been an increase in clinical awareness, which may be reflected in the increase in incidence.4 Despite calciphylaxis having a high morbidity and 1-year mortality as high as 80% in some cases,4 no randomized controlled trials examining longitudinal outcomes and treatment efficacy have been published.4 This report discusses 3 cases of early calciphylaxis diagnosis and treatment leading to rapid resolution and survival, highlighting the impact of timely recognition and intervention.

Calciphylaxis is characterized by calcific occlusion of vessels and subsequent tissue ischemia due to thrombosis. The precise pathogenetic mechanism behind calciphylaxis remains unclear. In the original experiment by Hans Selye and colleagues, soft-tissue calcification was induced in rats by applying a sensitizing agent, followed by a “challenger” agent after a specific time period. Trauma may represent one of these “challenger” agents, serving as an inducer of endothelial dysfunction and subsequent thrombosis, leading from tissue calcification to calciphylaxis. Koebnerization, a term used to describe the appearance of isomorphic lesions in areas of trauma, has been postulated to be a feature of calciphylaxis. This hypothesis arose from reports of patients who developed calciphylaxis following mild skin trauma, such as that caused by chronic resting of elbows on thighs, placement of ice packs, and injections involving various medications such as iron dextran, tobramycin, and especially insulin. Rigorous studies demonstrating the relationship between calciphylaxis and Koebnerization and an underlying mechanism are limited. To better understand this association, this study retrospectively identified characteristics of patients who presented with calciphylaxis in areas of trauma, suggesting the presence of Koebnerization.