Intravenous Immunoglobulin Dosing for Toxic Epidermal Necrolysis
For toxic epidermal necrolysis (TEN), the recommended dose of intravenous immunoglobulin (IVIG) is 2-3 g/kg total dose, administered as 1 g/kg daily for 3 consecutive days. 1
Evidence for IVIG Dosing in TEN
- High-dose IVIG (total dose of 2-3 g/kg) has been associated with improved survival compared to low-dose IVIG in adults with TEN 2
- A multicenter retrospective analysis of 48 consecutive TEN patients found that early infusion of IVIG at a total dose of 3 g/kg over 3 consecutive days (1 g/kg per day) was associated with rapid cessation of skin and mucosal detachment in 90% of patients and 88% survival 1
- Patients who responded better to IVIG had received treatment earlier in the course of disease and higher average doses 1
- Studies using lower doses (0.4 g/kg for 4 days) showed less favorable outcomes with mortality rates of 42% 2
Timing of Administration
- IVIG should be initiated as early as possible in the disease course, ideally within the first 24-48 hours of diagnosis 1, 3
- Delayed administration may reduce efficacy, with studies showing better outcomes when IVIG is started within the first 3 days of disease onset 4, 1
Duration of Treatment
- The standard duration is 3-5 consecutive days 2
- Most successful protocols administer the total dose over 3 days (1 g/kg/day) 1
- Some protocols have used 4-5 days with daily doses of 0.5-1.0 g/kg 4, 5
Efficacy Considerations
- There is significant batch-to-batch variation in the capacity of IVIG to inhibit Fas-mediated cell death in vitro, which may affect clinical efficacy 1
- The British Journal of Dermatology guidelines note that while some studies show benefit with high-dose IVIG, a meta-analysis found no overall survival benefit compared to supportive care alone (OR 1.00,95% CI 0.58-1.75) 2
- Pediatric patients treated with IVIG have significantly lower mortality than adults (0% vs. 21.6%) 2
Safety Profile
- IVIG is generally well-tolerated in TEN patients 4, 3
- No significant adverse reactions were reported in multiple studies using doses up to 3 g/kg 4, 1, 3
- IVIG may be safer than systemic corticosteroids, which carry concerns about increased infection risk 6
Important Caveats
- Despite promising results in some studies, the UK guidelines note that there is no active therapeutic regimen with unequivocal benefit for SJS/TEN 2
- The quality of evidence supporting IVIG use is limited (level 3-4 evidence) with most studies being retrospective or small case series 2
- High-quality supportive care remains the priority in TEN management regardless of specific interventions 6
- Consider combination therapy with corticosteroids in patients with high SCORTEN scores (≥2) as this may reduce hospitalization duration 7
Monitoring During Treatment
- Monitor for cessation of skin and mucosal detachment (typically occurs within 2-4 days of starting IVIG) 1, 3
- Assess for potential complications including thromboembolic events, renal dysfunction, and aseptic meningitis 6
- Continue treatment until clinical improvement is observed, typically for the full planned course 4, 5