What is the role of corticosteroids (CS) in the treatment of patients with Stevens-Johnson syndrome (SJS)?

Role of Corticosteroids in Stevens-Johnson Syndrome

The evidence does not support routine use of systemic corticosteroids in SJS/TEN, and high-quality multidisciplinary supportive care should be the priority. 1

Guideline Recommendations

The British Association of Dermatologists (2016) explicitly states there is no conclusive evidence to demonstrate the benefit of any one intervention over conservative management, and that high-quality, multidisciplinary supportive care is the priority. 1 The guideline development group found the data of insufficient quality or consistency to make specific recommendations either for or against corticosteroids (strength of recommendation D; level of evidence 4). 1

Key Evidence Limitations

• No randomized controlled trials exist - all available evidence comes from retrospective case series with significant methodological limitations. 1
• Major ascertainment bias exists in reported cohorts, with variations in timing and nature of intervention, case mix, setting, and quality of supportive care. 1
• Even among experienced clinicians managing SJS/TEN, there is lack of consensus on corticosteroid use. 1

Mixed Evidence on Efficacy

Potential Benefits (Weak Evidence)

• Retrospective EuroSCAR data showed lower mortality in German patients only (but not French patients) treated with corticosteroids compared to supportive care alone, highlighting geographic and methodological inconsistencies. 1, 2
• A meta-analysis including 96 studies and 3,248 patients suggested a survival benefit with glucocorticosteroids, but this was significant in only one of three statistical analyses. 1
• Small case series reported decreased mortality with high-dose pulse therapy: 12 patients receiving IV dexamethasone 100 mg or 1.5 mg/kg for 3 days had lower mortality than SCORTEN-predicted. 1
• Eight patients receiving IV methylprednisolone 1000 mg for 3 consecutive days had no deaths despite SCORTEN-predicted mortality of 1.6. 1

Significant Safety Concerns

The primary concern with systemic corticosteroids is increased infection risk, which is particularly dangerous in SJS/TEN patients who already have compromised skin barrier function. 1, 2

• A retrospective case series reported two deaths in patients treated with prednisolone. 1
• Corticosteroids may increase the risk of sepsis in this vulnerable population. 1
• The British Journal of Dermatology emphasizes that corticosteroids should be used with caution due to infection concerns. 1

Conflicting Data on Ocular Disease

There is conflicting data on whether systemic corticosteroids help limit ocular disease. 1, 2 One study showed no benefit of systemic corticosteroid use in acute SJS or TEN, while another showed significant improvement in best-corrected visual acuity and mean ocular involvement score in adults (but not in children) treated with corticosteroids or IVIg. 1

Alternative Treatments with Stronger Evidence

Ciclosporin

• Shows more promising evidence than corticosteroids: 29 patients with SJS/TEN receiving ciclosporin 3 mg/kg daily for 10 days (then tapered) had no deaths despite SCORTEN-predicted mortality of 2.75/29. 1, 2
• A meta-analysis reported no deaths and a regression model revealed significant beneficial effect compared with supportive care alone. 1

TNF-α Inhibitors

• Recent RCT shows promise for anti-TNF agents. 1
• One series of 10 patients treated with etanercept 50 mg subcutaneous had no deaths despite mean SCORTEN-predicted mortality of approximately 50%. 1

If Corticosteroids Are Used Despite Guidelines

Critical caveat: The following regimens are from case series only and should not be interpreted as endorsement. 2

• Methylprednisolone 40-80 mg daily (approximately 0.8-1.5 mg/kg/day) with rapid tapering over 7-10 days once disease progression arrests. 2
• IV dexamethasone 100 mg once daily for 3 days (though evidence is limited to 12 patients). 2
• Use the shortest possible duration with rapid tapering once disease progression arrests (typically 7-10 days total). 2

Critical Clinical Pitfalls to Avoid

• Do not use corticosteroids as monotherapy without considering alternatives like ciclosporin or IVIg, given the infection risk and lack of proven benefit. 2
• Do not delay supportive care while debating immunomodulatory therapy - supportive care is the proven priority. 1, 2
• If corticosteroids are initiated, monitor closely for infections with daily smears of skin and mucous membranes, as corticosteroids blunt the febrile response. 2
• The anti-inflammatory effects may not be apparent for 6-12 hours after administration, so do not expect immediate clinical improvement. 3

Special Considerations in Pediatric Populations

In children, individual case reports suggest early systemic glucocorticosteroids may limit disease progression, but there are no large studies documenting this benefit. 1 The same infection risk concerns apply, and the more favorable prognosis in pediatric TEN makes prevention of long-term complications the priority over aggressive immunosuppression. 1, 2