Abstract
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Introduction: Calcific uremic arteriolopathy, also known as calcific vasculopathy or calciphylaxis, is characterized by necrosis of the skin and fatty tissues. The cause is largely related to calcium deposition in soft tissues causing small blood vessel occlusion and skin necrosis. The disease is associated with renal failure and has high 1-year mortality rate of more than 50% (1). Diagnosis is based on clinical suspicion, imaging with radiographs, CT and bone scans, and tissue biopsy. This educational exhibit highlights the role of imaging modalities in early diagnosis and localization of this mortal disease.
Methods: We retrospectively evaluated referred cases to our department for bone scan with high clinical suspicion for calciphylaxis. Multi-imaging data was collected and their role in evaluation of the disease was assessed. Finally the histopathology results were used to confirm the calcific vasculopathy.
Results: Presented in the exhibit are picturesque illustration of calcific vasculopathy lesions (clinical presentation), evaluated on Bone scan, radiographs and CT scan. The cases include peripheral (truncal and appendicular) as well as visceral calciphylaxis and their pathology confirmation.
Conclusions: Calcific uremic arteriolopathy aka calcific vasculopathy is an uncommon and serious disease. Recent data shows about 1% of patients on dialysis being affected. Patients usually present with imbalance in calcium metabolism, with calcium deposition in the wall of the arterioles causing occlusion and necrosis of the overlying skin, leading to painful ulcers (2). Skin biopsy is the standard for diagnosis, however, can cause non-healing and worsening of the ulcers. Imaging modalities play an important role in localization and diagnosis formation. Radiographs demonstrate vascular calcification within the skin, however cannot differentiate from the renal patients without calciphylaxis. Bone scintigraphy demonstrate increased tracer uptake in calciphylaxis affected regions with enhanced uptake in indurated plaques. CT scan works similar to radiographs with higher resolution and may help in evaluation of visceral lesions. Treatment is directed towards normalizing the calcium and phosphate blood levels and meticulous wound management. Calcific vasculopathy is a challenging disease and early diagnosis is imperative for regulating calcium and improving overall quality of life for this subset of patients.
References:
Mazhar AR, Johnson RJ, Gillen D, et al. Risk factors and mortality associated with calciphylaxis in end-stage renal disease. Kidney Int. 2001;60(1):324–32. Mochel MC, Arakaki RY, Wang G, Kroshinsky D, Hoang MP. Cutaneous calciphylaxis: a retrospective histopathologic evaluation. Am J Dermatopathol. 2013;35(5):582–6.
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