Are intravenous (IV) steroids indicated for toxic epidermal necrolysis?

IV Steroids in Toxic Epidermal Necrolysis (TEN)

IV steroids are not recommended for toxic epidermal necrolysis due to conflicting evidence on efficacy and concerns about increased infection risk. 1

Evidence Assessment

Corticosteroid Use in TEN

• Systemic corticosteroids have historically been used in TEN management, with proponents emphasizing early administration to inhibit inflammation, but there is insufficient evidence to support their routine use 1
• Retrospective analysis of EuroSCAR data showed lower mortality in German patients (but not French patients) treated with corticosteroids compared to supportive care alone 1
• A meta-analysis including 96 studies and 3248 patients suggested a potential survival benefit with glucocorticosteroids, but this was significant in only one of three statistical analyses 1
• Two studies on pulsed IV and high-dose corticosteroids reported decreased mortality compared to SCORTEN predictions:
  • Kardaun's study (12 patients receiving 100 mg or 1.5 mg/kg IV dexamethasone for 3 days) 1
  • Hirahara's series (8 patients receiving 1000 mg IV methylprednisolone for 3 days) showed no deaths despite SCORTEN-predicted mortality of 1.6 1

Concerns with Corticosteroid Use

• There is significant concern that systemic corticosteroids may increase infection risk in TEN patients 1
• A retrospective case series reported two deaths in patients treated with prednisolone 1
• Some older literature considers steroids contraindicated in TEN 2
• In a retrospective study of 21 consecutive TEN patients, 40% (4/10) of steroid-treated patients died, compared to 36% (4/11) of non-steroid treated patients, suggesting no benefit 3

Alternative Treatment Options

• High-quality, multidisciplinary supportive care remains the priority in TEN management 1
• Other immunomodulatory therapies that have shown promise include:
  • Ciclosporin (3 mg/kg daily for 10 days, then tapered) showed effectiveness with no deaths despite SCORTEN-predicted mortality of 2.75/29 in one study 1
  • Intravenous immunoglobulin (IVIg) has shown mixed results, with some studies showing benefit and others showing no improvement in survival compared to supportive care 1
  • TNF-α inhibitors like etanercept have shown promise in recent studies, with one series of 10 patients showing no deaths despite a mean SCORTEN-predicted mortality rate of about 50% 1, 4
  • Combination therapy with low-dose IVIg and steroids has shown better outcomes than steroids alone in some studies 5

Special Considerations

• In pediatric populations, TEN has a lower mortality rate, so prevention of long-term complications becomes extremely important 1
• Ocular complications are common in TEN, and there is conflicting data on whether systemic corticosteroids help limit ocular disease 1
• Respiratory complications can be severe and may not correlate with the extent of epidermal detachment 1

Treatment Algorithm

1. First priority: Withdraw the causative drug and provide high-quality supportive care 1, 6
2. Consider alternatives to IV steroids:
   • Ciclosporin (3 mg/kg/day for 10 days, then tapered) 1
   • TNF-α inhibitors like etanercept (single 50-mg subcutaneous dose) 1, 4
   • IVIg (2 g/kg total dose) in selected cases 1
3. If steroids must be used (based on clinical judgment):
   • Use short course, high-dose regimen (e.g., IV dexamethasone 100 mg or 1.5 mg/kg for 3 days) 1
   • Monitor closely for infections 1
   • Consider combination with IVIg to potentially reduce mortality 5

Conclusion

Based on the most recent guidelines, IV steroids should not be routinely used in TEN due to conflicting evidence on efficacy and concerns about increased infection risk. High-quality supportive care remains the cornerstone of treatment, with ciclosporin and TNF-α inhibitors showing more promising results in recent studies.