Calciphylaxis (Calcific Uremic Arteriolopathy)

Figure 3.19 A: Calciphylaxis (Reprint from Morgan MB, Smoller BR, Somach SC. Deadly Dermatologic Diseases. New York, NY: Springer; 2007)

• Rare condition of systemic calcification involving small and medium-sized vessels with ischemic necrosis of the skin and soft tissue; high mortality
• Presents initially as painful violaceous mottling (reticulated) typically on lower extremities → stellate purpura with central necrosis, ± central bulla, followed by necrosis and ulceration; death due to gangrene and sepsis
• Most commonly in patients with end-stage renal disease on hemodialysis or after renal transplant with elevated calcium-phosphate product
• Treatment
  • Normalization of calcium-phosphate product (by low calcium dialysis)
  • Sodium thiosulfate (↑ solubility of calcium deposits)
  • Bisphosphonates (pamidronate, etridonate)
  • Calcimimetics (cinacalcet)
  • Parathyroidectomy
  • Wound therapy