Recommended Dose of Immunoglobulin for Toxic Epidermal Necrolysis
The recommended dose is 2-3 g/kg total, administered as 1 g/kg/day over 3 consecutive days, started as early as possible in the disease course. 1, 2
Dosing Algorithm
Standard High-Dose Protocol (Preferred)
- Total dose: 2-3 g/kg body weight 1, 2
- Administration schedule: 1 g/kg/day for 3 consecutive days 1, 2
- Timing: Initiate within 3 days of disease onset when possible 2
This high-dose regimen has demonstrated superior outcomes compared to lower-dose protocols, with mortality rates as low as 0-12% versus 42% with low-dose regimens (0.4 g/kg/day for 4 days). 3, 1
Alternative Dosing Regimens (Less Optimal)
Evidence Supporting High-Dose Therapy
The British Journal of Dermatology guidelines demonstrate that high-dose IVIG (2-3 g/kg total) is associated with improved survival compared to low-dose protocols. 1 A multicenter retrospective analysis of 48 patients showed 88% survival with mean total dose of 2.7 g/kg, with rapid cessation of disease progression (mean 2.3 days). 2
Studies using the 3-day high-dose protocol consistently report:
- Survival rates: 88-100% 6, 2, 5, 7
- Time to arrest disease progression: 2-3 days 6, 2, 5, 7
- Complete re-epithelialization: 7-8 days 5, 7
Critical Timing Considerations
Early initiation is crucial for efficacy. 2 Patients who responded to IVIG received treatment earlier in the disease course (mean 1.6-3.2 days from admission). 2, 5, 7 Delayed treatment beyond 8-10 days from disease onset may reduce effectiveness. 6
Important Caveats and Limitations
Despite promising case series data, the British Journal of Dermatology guidelines note that no active therapeutic regimen has unequivocal benefit for TEN, with only level 3-4 evidence supporting IVIG use. 3, 1 A meta-analysis found no overall survival benefit compared to supportive care alone (OR 1.00,95% CI 0.58-1.75). 1
However, the weight of evidence from multiple case series and the biological plausibility of blocking Fas-mediated apoptosis support high-dose IVIG as the most reasonable pharmacologic intervention when treatment is pursued. 2
Monitoring During Treatment
Monitor for IVIG-related complications including: 1
- Thromboembolic events (particularly with doses >2 g/kg)
- Renal dysfunction (especially in elderly or those with pre-existing renal disease)
- Aseptic meningitis
- Hemolytic anemia
Pediatric Considerations
Pediatric patients have significantly lower mortality (0% vs. 21.6% in adults) and respond well to the same weight-based dosing. 1, 7 The standard 2-3 g/kg total dose over 3 days is appropriate for children. 7
Common Pitfalls to Avoid
- Do not use low-dose protocols (0.4 g/kg/day for 4 days) - these have shown inferior outcomes with mortality rates up to 42%. 3, 1
- Do not delay treatment - efficacy decreases with later initiation beyond the first few days of disease. 2
- Do not assume all IVIG batches are equivalent - significant batch-to-batch variation exists in the capacity to inhibit Fas-mediated cell death. 2
- Do not rely solely on IVIG - high-quality multidisciplinary supportive care remains the foundation of TEN management. 1, 8