Calciphylaxis

Background

  • Most commonly seen in ESRD patients on hemodialysis (~1%)
    • Seen almost exclusively in patients with Stage 5 chronic kidney disease
  • No available data in general population (non-uremic calciphylaxis)
  • Calcium and phosphate levels rise beyond solubility and precipitate in arteries
  • May be increasing due to widespread IV vitamin D
  • Mortality as high as 60-80%; sepsis from necrotic skin lesions

Clinical Features

  • Very painful lesions develop suddenly and progress rapidly
  • Dermatolgic appearances:
    • Livedo reticularis
    • Stellate purpura
    • Usually lower extremities, hands, or torso

Differential Diagnosis

ESRD Associated Skin Conditions

Cardiovascular

Evaluation

Labs

  • Serum PTH level
  • CBC, CMP, phosphate, coags
  • Inpatient - hepatitis panel, cryofibrinogen level, lipase, ESR, CRP, ANA, ANCA

Imaging

  • Plain radiographs - arborization of vascular calcification within dermis and subQ tissues
  • Ultrasound may aid in examining for vascular calcification[1]

Biopsy[2]

  • Definitive means of diagnosis
  • Punch biopsy from lesion margin by dermatologist or wound surgeon
  • Caution in non-ulcerated/necrotic lesions as biopsy site has high likelihood of not healing in true calciphylaxis

Management

  • Rigorous and continuous control of phosphate and calcium balance
  • Medical
    • Discontinue calcium increasing interventions
    • Increase dialysis frequency
    • Calcimimetics in hyperparathyroidism
    • Bisphosphonates
    • Sodium thiosulfate - off-label, increases solubility of calcium deposits
    • Fix hypercoagulability
    • Surgical
  • Aggressive wound care and debridement of necrotic tissues
    • Wound VAC
    • Total or subtotal parathyroidectomy

Disposition

  • Admit

See Also

References

  1. Bukhman R et al. Sonography in the Identification of Calciphylaxis of the Breast. JUM January 1, 2010 vol. 29 no. 1 129-133.
  2. Nigwekar SU et al. Calciphylaxis: Risk Factors, Diagnosis, and Treatment. Am J Kidney Dis. 2015;66(1):133-146.