Calciphylaxis

A 66-year-old woman with a history of diabetes, hypertension, and chronic kidney disease, not on maintenance haemodialysis, was admitted to our emergency department with complaints of fever, bilateral lower limb pain, and behavioural changes. She was diagnosed with uraemia and was shifted to the intensive care unit for initiation of haemodialysis. During admission, she developed a painful purpuric rash on her right thigh and left leg. A few days later, the rash progressed to form a necrotic ulcer (Fig. 1). A non-contrast CT scan showed calcification of the walls of the abdominal aorta and its branches (Fig. 2). A biopsy (Fig. 3) of the skin lesions showed calcification in dermal and subcutaneous micro vessels with septal panniculitis, confirming the diagnosis of calciphylaxis.

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FIG 1.

A necrotic eschar involving the right thigh with surrounding areas of desquamation

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FIG 2.

Coronal CT scan of the abdomen and pelvis showing extensive calcification along the abdominal aorta and iliac arteries (blue arrow), with heavy mural calcification extending into the right common femoral artery (green arrow)

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FIG 3.

Microscopic appearance of calciphylaxis in subcutaneous adipose tissue with calcification of small- and medium-sized arterioles (black arrows), concentric medial calcification and luminal narrowing, along with surrounding fat necrosis and haemorrhage in the adipose lobules (red arrows); H&E stain

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Calciphylaxis is a life-threatening syndrome of vascular calcification characterized by microvessel thrombosis and ischaemia of the subcutaneous adipose tissue and dermis that results in painful skin lesions.¹ Currently, there are no approved treatments for calciphylaxis.² She was started on sodium thiosulphate injections, which were given during the last hour of the dialysis session. However, the lesions did not improve with treatment.