Calciphylaxis due to hyperparathyroidism.

OBJECTIVE: To discuss when and how to workup calciphylaxis for early diagnosis and to delineate medical vs surgical management of the disease.
METHODS: Review of evidence-based medical literature on calciphylaxis due to primary, secondary, and tertiary hyperparathyroidism.
RESULTS: Calciphylaxis is usually associated with secondary and tertiary hyperparathyroidism. However, calciphylaxis can also be seen in the absence of chronic renal failure and has been reported in patients with primary hyperparathyroidism due to a parathyroid adenoma or carcinoma. Calciphylaxis occurs when the levels of calcium and phosphate in the blood exceed their solubility level, leading to calcium-phosphate deposits in arteries that compromise the vasculature. These ischemic changes result in plaque-like lesions that progress to painful nodules. Calciphylaxis is diagnosed on the basis of physical examination, laboratory, and histopathologic findings. When medical therapy has failed in the setting of secondary/tertiary hyperparathyroidism with calciphylaxis, parathyroidectomy is the preferred treatment. In primary hyperparathyroidism, early recognition and aggressive wound care with debridement are important in managing this condition. However, resection of the offending parathyroid gland should be strongly considered.
CONCLUSIONS: When calciphylaxis is present, early detection is critical to the successful management of this condition. Although medical therapy can be effective, surgical resection of the diseased parathyroid glands can be curative and potentially life saving. A multidisciplinary approach involving early diagnosis, aggressive medical management, operative debridement, and parathyroidectomy has the best chance of improving survival in calciphylaxis.