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

Abstract

Calciphylaxis (CP) is a rare disorder presenting with painful ischemic skin ulcerations. Its association with end-stage renal disease, but also non-uremic cases have been described. Several risk factors, like metabolic syndrome, calcium-phosphate imbalance, anticoagulative medication and female gender have been discussed. Multidisciplinary therapeutic approaches are necessary since evidence-based guidelines are missing due to lack of pathophysiological understanding of the disease and the absence of studies with large patient cohorts. However, strategies for local wound therapy, reduction of risk factors and systemic therapies are being developed. Changes in duration, frequency and method of hemodialysis and specific medication to lower the calcium upload can be effective in uremic CP cases. Systemic treatments with cinacalcet, sodium thiosulfate or bisphosphonates have been applied successfully in CP patients, but large placebo-controlled randomized trials are still needed to gather better insight into this fatal disease.

Occlusive cutaneous vasculopathies as cause of chronic ulcers

“…There have, however, been reports of an association between calciphylaxis and coagulation disorders; so this should be investigated. 61 More specific laboratory parameters include fetuin A, sclerostin, osteoprotegerin, TRAP5b, bone-specific alkaline phosphatase, serum cystatin C, globular arrest protein I, or fibroblast growth factor 23. These, however, mainly possess an experimental value and are not important for routine diagnostics.…”

“…These, however, mainly possess an experimental value and are not important for routine diagnostics. 61 Histological examination completes the diagnostic workup. This requires a deep, spindleshaped skin biopsy.…”

“…To exclude potential differential diagnoses, additional analyses of coagulation factors and auto‐antibodies should be performed, depending on the suspected differential diagnosis. There have, however, been reports of an association between calciphylaxis and coagulation disorders; so this should be investigated 61 . More specific laboratory parameters include fetuin A, sclerostin, osteoprotegerin, TRAP5b, bone‐specific alkaline phosphatase, serum cystatin C, globular arrest protein I, or fibroblast growth factor 23.…”

“…More specific laboratory parameters include fetuin A, sclerostin, osteoprotegerin, TRAP5b, bone‐specific alkaline phosphatase, serum cystatin C, globular arrest protein I, or fibroblast growth factor 23. These, however, mainly possess an experimental value and are not important for routine diagnostics 61 . Histological examination completes the diagnostic workup.…”

Management of Cutaneous Calciphylaxis

“…As an adjunctive treatment, hyperbaric oxygen therapy has been shown to achieve 58% wound improvement and 50% wound resolution in uremic calciphylaxis [18,21]. It is important to promote optimal healing via nutritional management, correction of anemia, and optimization of dialysis parameters [20,22].…”