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.

Background: Calciphylaxis is an ischemic vasculopathy with high morbidity and mortality. Early and accurate diagnosis is critical to management of calciphylaxis. Clinical mimickers may contribute to delayed or misdiagnosis. Objective: To assess the rate and risk factors for misdiagnosis and to identify clinical mimickers of calciphylaxis. Methods: A retrospective medical record review was conducted of patients with calciphylaxis at a large urban tertiary care hospital between 2006 and 2018. Results: Of 119 patients diagnosed with calciphylaxis, 73.1% were initially misdiagnosed. Of patients not initially misdiagnosed, median time to diagnosis from initial presentation was 4.5 days (interquartile range, 1.0-23.3), compared to 33 days (interquartile range, 13.0-68.8) in patients who were initially misdiagnosed (P = .0002). The most common misdiagnoses were cellulitis (31.0%), unspecified skin infection (8.0%), and peripheral vascular disease (6.9%). Patients who were misdiagnosed frequently received at least 1 course of antibiotics. Patients with end-stage renal disease were less likely to be misdiagnosed than those without this disease (P = .001). Limitations: Single-center, retrospective study. Conclusions: Understanding the risk factors for misdiagnosis of calciphylaxis is an opportunity for further education concerning this rare disease.

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.

BACKGROUND: Calciphylaxis is a rare disorder characterized by skin necrosis caused by calcium deposition within vessels, thrombosis, and subsequent tissue ischemia. Penile involvement may rarely occur. OBJECTIVE: To identify risk factors, diagnosis, management, and mortality of patients with penile calciphylaxis. METHODS: A retrospective medical record review was conducted of 16 patients with penile calciphylaxis treated at 2 large urban tertiary care centers between January 2001 and December 2019. A control group of 44 male patients with nonpenile calciphylaxis at the same institution was included. RESULTS: The median survival of patients with penile calciphylaxis was 3.8 months (interquartile range, 27.0 months). Mortality was 50% at 3 months and 62.5% at 6 months for penile calciphylaxis, and 13.6% at 3 months and 29.5% at 6 months for controls (P = .008). Patients with penile calciphylaxis were less likely to be obese (P = .04) but more likely to have hyperparathyroidism (P = .0003) and end-stage renal disease (P = .049). LIMITATIONS: Retrospective study design and small sample size. CONCLUSIONS: This study further defines the disease course of penile calciphylaxis, which has high mortality. Imaging may be used to aid diagnosis. Risk factors include end-stage renal disease, hyperparathyroidism, and normal body mass index.