Calciphylaxis in Patients With Preserved Kidney Function

Abstract

Calcific uremic arteriolopathy (CUA), also known as calciphylaxis, is a devastating disease typically seen in patients with end stage renal disease. It manifests as extremely painful symmetrical wounds resistant to surgical and medical interventions. The prevalence of CUA among hemodialysis dependent patients was found to be as high as 4.1%. The management of patients with CUA requires a multidisciplinary approach by the medical team, yet often results in a low rate of successful outcomes. Recently, non-uremic calciphylaxis (NUC) has been described in the absence of kidney disease. Limited knowledge exists on the management of NUC and the outcomes of this condition. Herein we describe three clinical scenarios of patients diagnosed with NUC in the absence of permanent or prolonged acute renal pathology. The reporting of successful and fruitless therapeutic interventions for wound management in NUC is important for compiling the evidence of effective therapeutic strategies.

Keywords: Non-uremic calciphylaxis; Sodium thiosulfate; Wound healing.

Figure 1

Initial presentation of right thigh calciphylaxis. Painful erythematous and purplish ulcer with hard subcutaneous lumps representing the fat necrosis.

Figure 2

Histology showing linear thick calcification of the media of the arterioles seen on biopsy.

Figure 3

Histology showing calcification of the media of the arterioles, intimal hyperplasia and intraluminal red blood cells.

Figure 4

An advanced stage of the lesion after surgical excisional debridement of the necrotic soft tissue including skin and subcutaneous fatty tissue on the right thigh and buttock.