Systemic Corticosteroids in Stevens-Johnson Syndrome
The 2016 British Journal of Dermatology guideline explicitly recommends against routine use of systemic corticosteroids in Stevens-Johnson syndrome due to insufficient evidence of benefit and significant infection risk; however, if corticosteroids are used, early high-dose intravenous methylprednisolone (0.5–1 mg/kg) initiated within 72 hours of onset may be considered in selected cases, with cyclosporine 3 mg/kg daily representing a superior alternative with stronger supporting evidence. 1, 2
Guideline Position on Corticosteroids
The highest-quality guideline evidence (British Journal of Dermatology, 2016) states there is no conclusive evidence that any active intervention, including corticosteroids, improves outcomes over conservative management alone (strength of recommendation D, level of evidence 4). 1, 3
No randomized controlled trials exist for corticosteroid use in SJS—all available data derive from retrospective case series with major ascertainment bias and methodological limitations. 1, 3
The primary concern driving this recommendation is that systemic corticosteroids significantly increase infection risk in patients who already have compromised skin barrier function, and infection is the leading cause of death in SJS/TEN. 1, 3
Evidence Supporting Limited Corticosteroid Use
Retrospective EuroSCAR data showed lower mortality in German patients (but not French patients) treated with corticosteroids compared to supportive care alone, highlighting geographic inconsistencies and selection bias. 1, 3
Small case series using high-dose pulsed regimens (dexamethasone 100 mg daily for 3 days or methylprednisolone 1000 mg daily for 3 days) reported mortality lower than SCORTEN-predicted rates. 3, 4
One uncontrolled series of 12 patients treated with dexamethasone pulse therapy had 1 death versus 4 predicted by SCORTEN, with stabilization in 2.3 days and re-epithelialization in 13.9 days. 4
Two older case series (54 and 13 patients) reported no SJS-related deaths with corticosteroid therapy, but these lacked control groups and SCORTEN validation. 5, 6
A 2016 retrospective analysis of 70 patients showed actual mortality significantly lower than SCORTEN-predicted mortality (SMR = 0.30, P = 0.0168), particularly in the low-dose corticosteroid group (SMR = 0.20, P = 0.0145). 7
If Corticosteroids Are Used Despite Guidelines
Timing is critical: Corticosteroids must be initiated within 72 hours of disease onset to have any potential benefit. 1, 2
Dosing regimen:
- Intravenous methylprednisolone 0.5–1 mg/kg daily (or oral prednisolone 1–2 mg/kg/day if IV access unavailable). 2
- Continue for the shortest duration possible—typically until disease progression arrests (usually 2–5 days). 1
- Transition to oral corticosteroids with a rapid taper over at least 4 weeks total. 2
Alternative pulse regimen:
- Dexamethasone 100 mg IV once daily for 3 days, or methylprednisolone 1000 mg IV daily for 3 days. 3, 4
Superior Alternative: Cyclosporine
Cyclosporine is the preferred immunomodulatory agent when supportive care alone is inadequate, based on stronger evidence than corticosteroids. 1, 3
Dosing: 3 mg/kg per day (divided into two doses) for 10 days, followed by a taper over approximately one month. 1, 3
Evidence of benefit: A landmark prospective study of 29 patients treated with cyclosporine had 0 deaths despite SCORTEN-predicted mortality of 2.75 deaths (approximately 9.5% predicted vs. 0% actual). 1, 3
Mechanistic advantage: Cyclosporine directly inhibits CD8+ T-cell signaling that drives keratinocyte apoptosis in SJS, without the broad immunosuppression and infection risk of corticosteroids. 3
Critical Management Priorities (Universal Recommendations)
Immediate drug discontinuation: Stop all suspected culprit medications immediately—this is the single most critical intervention. 1, 2
Transfer to specialized care: Patients with >10% body surface area involvement must be transferred to a burn center or ICU within hours; delayed transfer markedly increases mortality. 1, 2
SCORTEN calculation: Calculate within 24 hours to predict mortality risk (scores 0–7 correspond to 1%–99% mortality) and guide intensity of care. 1, 2
Ophthalmology consultation: Mandatory within 24 hours of diagnosis; failure to do so leads to permanent visual impairment. 1, 2
Infection prevention: Do not use prophylactic antibiotics—they promote resistant organism colonization (especially Candida) without improving outcomes. 1, 2
Targeted antimicrobials: Initiate antibiotics only when clinical infection signs appear (confusion, hypotension, oliguria, desaturation, increased skin pain, rising CRP, neutrophilia). 1, 2
Special Considerations
Immune checkpoint inhibitor–induced SJS: In this specific context, corticosteroids are not contraindicated because the pathology is T-cell–mediated immune toxicity rather than drug hypersensitivity. 2
- Grade 3 disease: methylprednisolone 0.5–1 mg/kg IV. 2
- Grade 4 disease: methylprednisolone 1–2 mg/kg IV. 2
- Permanently discontinue the checkpoint inhibitor. 2
Pediatric patients: Mortality is lower in children (0–8.5%), but infection triggers (especially Mycoplasma pneumoniae) account for up to 50% of cases—test for infectious causes and involve infectious disease specialists. 2
Critical Pitfalls to Avoid
Do not use corticosteroids as monotherapy without considering cyclosporine, given the lack of proven benefit and heightened infection risk. 1
Do not delay transfer to specialized care while debating immunomodulatory options—timely multidisciplinary supportive care saves more lives than any specific drug. 1
Do not employ prophylactic antibiotics even when corticosteroids are administered, as this compounds infection risk. 1, 2
Do not continue corticosteroids beyond the acute progression phase—prolonged use increases infection risk without additional benefit. 8
Do not use corticosteroids if presentation is delayed beyond 72 hours—there is no evidence of benefit after this window. 1, 2