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.