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Calciphylaxis
From WikEM
Contents
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
- Brown recluse spider bite
- Bullous pemphigoid
- Cellulitis, necrotizing fasciitis
- Erythema nodosum
- Vasculitis
- Venous ulcers
- Hypercalcemia
- Hyperphosphatemia
ESRD Associated Skin Conditions
- Calciphylaxis
- Nephrogenic Systemic Fibrosis (gadolinium MRI)
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
- ↑ Bukhman R et al. Sonography in the Identification of Calciphylaxis of the Breast. JUM January 1, 2010 vol. 29 no. 1 129-133.
- ↑ Nigwekar SU et al. Calciphylaxis: Risk Factors, Diagnosis, and Treatment. Am J Kidney Dis. 2015;66(1):133-146.
Authors
Kevin Lu, Ross Donaldson, Michael Holtz, Daniel Ostermayer, Neil Young
