Calciphylaxis in a patient with hypoparathyroidism and MEN-1 syndrome.

Abstract

Summary: Calciphylaxis is a rare disorder characterised by the development of painful necrotic skin lesions. Occlusion of cutaneous arterioles due to ectopic calcification leads to potentially life-threatening widespread skin loss. Most cases occur in patients with chronic renal disease, which leads to dysregulation of calcium and phosphate homeostasis. Only a handful of case reports exist describing calciphylaxis occurring in patients without chronic renal disease but with hypoparathyroidism. We report on a unique case of a 53-year-old man with multiple endocrine neoplasia type 1 syndrome and acquired hypoparathyroidism due to total parathyroidectomy who went on to develop calciphylaxis following cardiac surgery.

Learning points: Calciphylaxis most commonly occurs in the context of chronic renal disease but can rarely occur in its absence as a consequence of calcium and phosphate dysregulation. Patients who develop necrotic skin lesions in the presence of hypoparathyroidism require an urgent dermatological opinion. Mortality from calciphylaxis is high, with the majority of deaths occurring secondary to sepsis. Management of calciphylaxis requires a multidisciplinary team approach to manage wound healing, infections and pain. Recovery with full rehabilitation from calciphylaxis can take months to years.