Calciphylaxis and Mönckberg’s Arteriosclerosis in a Diabetic Patient

Abstract

Diffuse arterial calcification is characteristic of peripheral arterial disease (PAD), particularly in diabetic patients. In diabetic patients, the rates of PAD and the more advanced forms of vascular disease such as critical limb ischemia are higher, and vascular intervention is known to be more difficult and less effective. For 1 in 6 patients, below-knee amputations can result from microvascular disease without traditional PAD. Microvascular disease occurs in vessels with a diameter around 100 µm and is most commonly present in diabetic patients. Calciphylaxis is a life-threatening complex disorder that presents with painful ischemic skin lesions caused by occlusion of blood vessels in subcutaneous fat and dermis. Mönckberg’s arteriosclerosis results in extensive calcium deposits in the tunica media without obstruction into the lumen. This case highlights a young diabetic patient with rapidly progressing acute onset ulcers of the bilateral lower extremities status post beginning peritoneal dialysis. The patient underwent upper and lower arterial noninvasive studies, angiogram, computerized tomography angiography, and punch biopsy. The arterial duplex examinations were normal with the exception of noncompressible calcified vessels. Microvascular disease was correlated with angiogram. Computed tomography angiogram findings were conclusive for calciphylaxis and Mönckberg’s arteriosclerosis. Punch biopsy was performed to confirm calciphylaxis and patient was started on the appropriate treatment regimen. Unfortunately, limb salvage was not possible and amputations were ultimately performed. There are other vascular pathologies that are not associated with traditional large artery atherosclerosis, such as calciphylaxis and Mönckberg’s arteriosclerosis. Even with seemingly normal arterial testing, the prognosis can be catastrophic. Although rare, these additional disease processes should be considered in dialysis patients with acute ulcerations.