Is it recommended to use systemic corticosteroids (steroids) in patients with Toxic Epidermal Necrolysis (TEN) who are receiving intravenous immunoglobulin (IVIG)?

Systemic Corticosteroids in TEN Patients Receiving IVIG

Systemic corticosteroids are not recommended for routine use in patients with Toxic Epidermal Necrolysis (TEN) who are receiving intravenous immunoglobulin (IVIG) due to conflicting evidence on efficacy and concerns about increased infection risk. 1

Evidence Assessment for Corticosteroid Use in TEN

• The British Journal of Dermatology guidelines indicate that systemic corticosteroids have historically been used in TEN management, 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 2
• Some studies have investigated pulsed IV and high-dose corticosteroids with reports of decreased mortality compared to SCORTEN predictions, but these are limited by study design and small sample sizes 2
• A retrospective study found no significant difference in mortality between patients treated with corticosteroids (40% mortality) and those not treated with steroids (36% mortality) 3

Concerns with Corticosteroid Use

• There is significant concern that systemic corticosteroids may increase infection risk in TEN patients 1
• Sepsis is a common complication in TEN patients, with one study reporting 18 out of 21 patients developing sepsis with various organisms including Enterococcus, Acinetobacter, and Staphylococcus aureus 3
• The presence of neutropenia, renal impairment, and disseminated intravascular coagulation were identified as strong risk factors for mortality in TEN patients 3

IVIG Treatment Considerations

• 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 4
• Studies using lower doses (0.4 g/kg for 4 days) showed less favorable outcomes with mortality rates of 42% 4
• The standard duration of IVIG treatment is 3-5 consecutive days, with most successful protocols administering the total dose over 3 days (1 g/kg/day) 4

Combined Therapy Considerations

• A propensity-matched retrospective study from China found that patients treated with IVIG combined with corticosteroids had a 45% lower mortality rate than those treated with corticosteroids alone, although this was not statistically significant 5
• The same study reported that combination therapy was associated with significantly lower incidence of skin infections (p<0.025) and a 67% decrease in total infection rate compared to corticosteroids alone 5
• Another Chinese study showed that IVIG combined with corticosteroids demonstrated a trend toward reducing mortality (13%, SMR=52.35) compared to corticosteroid monotherapy (31%, SMR=123.92) 6
• A Japanese study reported that IVIG (400 mg/kg per day for 5 days) administered as additional therapy to systemic steroids showed efficacy in 87.5% of patients with SJS or TEN who did not respond sufficiently to systemic steroids alone 7

Alternative Treatment Options

• High-quality, multidisciplinary supportive care remains the priority in TEN management regardless of specific interventions 1
• Ciclosporin has shown promise in recent studies, with one study reporting no deaths despite a SCORTEN-predicted mortality of 2.75/29 when using ciclosporin at 3 mg/kg daily for 10 days, then tapered 1, 8
• TNF-α inhibitors have also shown potential benefit in TEN management 1

Clinical Decision Algorithm

1. Prioritize high-quality supportive care for all TEN patients 1
2. For patients already receiving IVIG:
   • Continue IVIG at high dose (total 2-3 g/kg over 3-5 days) 4
   • Avoid adding systemic corticosteroids due to increased infection risk 1, 3
3. If additional immunomodulatory therapy is needed:
   • Consider ciclosporin (3 mg/kg daily for 10 days, then tapered) as an alternative to corticosteroids 1, 8
4. Monitor closely for complications, particularly:
   • Sepsis and other infections 3
   • Neutropenia, renal impairment, and coagulopathies 3
   • Ocular complications 2

Special Considerations

• Ocular complications are common in TEN, and there is conflicting data on whether systemic corticosteroids help limit ocular disease 1
• In patients with significant ocular involvement, early ophthalmology consultation is essential, and topical corticosteroid drops (e.g., nonpreserved dexamethasone 0.1%) supervised by an ophthalmologist may be considered 2
• Respiratory complications can be severe and may not correlate with the extent of epidermal detachment 1