Calciphylaxis is a rare syndrome of vascular calcification with thrombosis that occurs most often in patients with end stage renal disease, and it frequently portends a guarded prognosis. Rarely, non-uremic calciphylaxis may occur; in this context, a strongly supportive histology is crucial in establishing the diagnosis. Herein, we describe two cases of non-uremic calciphylaxis associated with pseudoxanthoma elasticum-like changes, identified in both initial non-diagnostic and subsequent diagnostic biopsy specimens. This unusual but helpful histologic finding may support the early diagnosis and treatment of a potentially life-threatening disease in the context of subtle histopathologic vascular changes or in the absence of classic clinical or laboratory findings.
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