Are systemic corticosteroids recommended for Stevens-Johnson syndrome, and if so, what is the appropriate dosing regimen?

Systemic Corticosteroids in Stevens-Johnson Syndrome

The British Association of Dermatologists recommends against routine use of systemic corticosteroids in Stevens-Johnson syndrome due to insufficient evidence of benefit and significant infection risk, though early high-dose methylprednisolone (within 72 hours) may be considered in selected cases. 1, 2

Evidence Assessment

The controversy over corticosteroid use in SJS reflects fundamentally weak evidence:

• No randomized controlled trials exist—all available data come from retrospective case series with major methodological limitations and ascertainment bias 1, 3, 4
• A meta-analysis of 96 studies (3,248 patients) suggested survival benefit with glucocorticosteroids, but this was significant in only one of three statistical analyses 1, 5
• Retrospective EuroSCAR data showed lower mortality in German patients treated with corticosteroids compared to supportive care alone, but this benefit was not observed in French patients, highlighting geographic inconsistencies 2, 3
• The UK guideline carries a strength of recommendation D and level of evidence 4, explicitly stating there is no conclusive evidence that corticosteroids improve outcomes over conservative management 3

Primary Safety Concern: Infection Risk

Systemic corticosteroids may increase infection risk in patients who already have compromised skin barrier function, which is particularly dangerous given that sepsis is a leading cause of mortality in SJS/TEN 1, 2, 3:

• A retrospective case series reported two deaths in patients treated with prednisolone 1
• Corticosteroids blunt the febrile response, making infection detection more difficult 2
• The British Association of Dermatologists emphasizes that corticosteroids should be used with extreme caution due to these infection concerns 1, 3

If Corticosteroids Are Used Despite Guidelines

When clinicians elect to use corticosteroids (recognizing this is off-label and not routinely recommended), the regimen should be:

• Intravenous methylprednisolone 0.5–1 mg/kg (or oral prednisolone 1–2 mg/kg/day) initiated within 72 hours of disease onset 2, 6
• Transition to oral corticosteroids with a taper lasting at least 4 weeks 2, 6
• Use the shortest possible duration with rapid tapering once disease progression arrests (typically 7–10 days total) 2
• Do not use corticosteroids as monotherapy without considering alternatives like cyclosporine 2

Preferred Alternative: Cyclosporine

Cyclosporine has stronger supporting evidence than corticosteroids and should be considered first-line immunomodulatory therapy when supportive care alone is inadequate 2, 3:

• Dosage: 3 mg/kg per day (divided into two doses) for 10 days, followed by a taper over approximately one month 2, 3
• A landmark prospective study of 29 patients treated with cyclosporine reported 0 deaths despite SCORTEN-predicted mortality of 2.75/29 1, 2, 3
• Meta-analysis showed significant beneficial effect compared with supportive care alone 1
• Mechanistic advantage: cyclosporine directly inhibits CD8+ T-cell signaling that drives keratinocyte apoptosis, without the broad immunosuppression of corticosteroids 3

Special Consideration: Immune Checkpoint Inhibitor–Induced SJS

In the specific context of immune checkpoint inhibitor–induced SJS/TEN, corticosteroids are NOT contraindicated because the pathology is driven by T-cell–mediated immune toxicity rather than infection 6:

• Grade 4 disease: methylprednisolone 1–2 mg/kg IV 6
• Grade 3 disease: methylprednisolone 0.5–1 mg/kg IV 6
• Permanently discontinue the offending immune checkpoint inhibitor 6

Core Management Priorities (Universal Recommendations)

Regardless of immunomodulatory therapy chosen, high-quality multidisciplinary supportive care remains the definitive priority 2, 3:

• Immediate discontinuation of all suspected culprit drugs is the single most critical intervention 2, 6
• Transfer to specialized burn center or ICU within hours for patients with >10% body surface area involvement; delayed transfer markedly increases mortality 2, 6
• SCORTEN calculation within 24 hours to estimate mortality risk (scores 0–7 correspond to 1%–99% predicted mortality) 2, 6
• Ophthalmology consultation within 24 hours is mandatory; failure to do so is associated with permanent visual impairment 2, 6

Critical Pitfalls to Avoid

• Do not use prophylactic systemic antibiotics even when corticosteroids are administered—this promotes resistant organism colonization (especially Candida) without improving outcomes 2, 6
• Do not delay transfer to specialized care while debating immunomodulatory options; timely multidisciplinary supportive care saves more lives than any specific drug 2
• Monitor closely for infections with daily skin swabs if corticosteroids are used, as they mask fever and other infection signs 2
• Do not continue corticosteroids beyond the acute progression phase—withdraw as soon as disease stabilizes to minimize infection risk 2, 7

Pediatric Considerations

In children, mortality is lower than adults (0–8.5% depending on severity), making prevention of long-term complications the priority over aggressive immunosuppression 1, 6:

• Individual case reports suggest early glucocorticosteroids may limit disease progression, but no large studies document this benefit 1
• The same infection risk concerns apply to pediatric patients 1
• Up to 50% of pediatric SJS/TEN cases are triggered by infections (Mycoplasma pneumoniae, HSV); test for infective triggers and involve infectious disease specialists 6