Hardening of Skin in Hemodialysis Patient: Calciphylaxis Management
Immediate Recognition and Diagnosis
This is calciphylaxis (calcific uremic arteriolopathy) until proven otherwise, and requires urgent multidisciplinary intervention given the 50-80% one-year mortality rate. 1, 2
Key Diagnostic Features to Confirm
- Painful, violaceous, mottled skin lesions progressing to non-healing ulcers and necrosis, typically affecting lower extremities symmetrically 3
- Skin biopsy showing microvascular calcification with endovascular fibrosis and thrombosis in small-to-medium dermal vessels 2, 4
- CT imaging demonstrating extensive arterial calcifications in affected areas 2
- Sepsis from superinfection is the leading cause of death, occurring in >50% within the first year 1
Treatment Algorithm
Step 1: Optimize Dialysis Parameters (Critical First Intervention)
Intensify hemodialysis prescription immediately to achieve target Kt/V of approximately 1.6 or higher 5, 4
- Increase dialysis frequency and/or duration beyond standard prescription 5
- Consider high-flux hemodialysis over standard filtration 6
- This addresses the fundamental uremic state driving the pathology 5
Step 2: Correct Mineral Metabolism Abnormalities
Normalize calcium-phosphate balance and control parathyroid hormone levels 7, 4
- Discontinue all calcium-based phosphate binders immediately 7
- Stop vitamin D supplementation 2
- Discontinue iron supplementation 2
- Use dialysate calcium of 1.50 mmol/L or higher to maintain neutral calcium balance while avoiding hypercalcemia 7
- Add phosphate dialysate additive if hypophosphatemia develops after stopping binders 7
Step 3: Medication Review and Adjustments
Stop warfarin immediately if patient is anticoagulated 4
- Warfarin is a known risk factor for calciphylaxis progression 4
- Consider alternative anticoagulation strategies if absolutely necessary
Step 4: Initiate Sodium Thiosulfate Therapy
Administer sodium thiosulfate with each hemodialysis session 2, 5
- This is the primary pharmacologic intervention with evidence for calciphylaxis 2, 5
- Continue throughout treatment course 5
Step 5: Aggressive Wound Management
Implement diligent wound care with multidisciplinary wound team 5, 4
- Debridement of necrotic tissue as appropriate 4
- Targeted antibiotics for secondary infection based on wound cultures 4
- Monitor closely for sepsis development, which is the primary cause of mortality 1, 2
Critical Pitfalls to Avoid
- Do not delay diagnosis: Early recognition is crucial as mortality increases dramatically once ulceration occurs (80% one-year mortality with ulcers) 2
- Do not continue calcium-based binders: These worsen calcium-phosphorus dysregulation 7, 5
- Do not maintain standard dialysis prescription: Inadequate dialysis perpetuates the uremic state 5
- Do not miss sepsis: Monitor vigilantly for infection as this is the most common cause of death 1, 2
- Do not confuse with cellulitis: Patients often have multiple prior admissions misdiagnosed as cellulitis before calciphylaxis is recognized 1
Expected Clinical Course
- Response to therapy is often poor despite aggressive intervention 2
- Wounds may progress significantly over 8 weeks even with optimal treatment 2
- Prolonged healing complicated by secondary infection is common 4
- Some patients achieve successful management with the integrated approach described, particularly when caught early 5
- Mortality within 2 months of diagnosis is not uncommon in severe cases 1