Treatment of Stevens-Johnson Syndrome with IVIG

Primary Treatment Approach

The American College of Oncology recommends discontinuing the culprit drug immediately and admitting to a burn unit or ICU with dermatology consultation for severe Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) 2, 3
Provide meticulous supportive care including fluid/electrolyte balance, wound care, and infection prevention for patients with SJS/TEN 2, 3
Apply topical emollients, high-strength topical corticosteroids, and oral antihistamines for patients with SJS/TEN 2, 3

For Grade 3-4 SJS/TEN, first-line treatment is IV methylprednisolone 0.5-1 mg/kg for Grade 3, or 1-2 mg/kg for Grade 4, tapering when toxicity resolves 2, 3
IVIG or cyclosporine may be added in severe or steroid-unresponsive cases of SJS/TEN 2, 3

High-dose IVIG regimen (2-3 g/kg over 3-5 days) is preferred for treating SJS/TEN, with most successful protocols using 1 g/kg/day for 3 days 1
Low-dose IVIG regimen (0.4 g/kg for 4 days) shows inferior outcomes with mortality rates of 42% 1

No overall survival benefit was demonstrated in meta-analysis comparing IVIG to supportive care alone for SJS/TEN (OR 1.00, 95% CI 0.58-1.75) 1
High-dose IVIG (2-3 g/kg) has been associated with improved survival compared to low-dose regimens in adults with SJS/TEN 1
Pediatric patients treated with IVIG have significantly lower mortality than adults (0% vs. 21.6%) 1

Severe disease (Grade 4) with >10% BSA involvement and systemic symptoms is an indication for IVIG use 2, 3
Steroid-unresponsive cases after initial corticosteroid therapy are an indication for IVIG use 2, 3
Rapidly progressive disease despite supportive care is an indication for IVIG use 1
Pediatric patients are an indication for IVIG use given their superior outcomes 1

Monitor for thromboembolic events, renal dysfunction, and aseptic meningitis during IVIG treatment 1
IVIG may be safer than systemic corticosteroids alone, which carry increased infection risk 1, 4

For immune checkpoint inhibitor-induced SJS/TEN, the usual prohibition of corticosteroids does not apply, and adequate immunosuppression is required 2, 3
UK guidelines note insufficient evidence to recommend IVIG routinely, suggesting it should only be administered under specialist supervision in the context of clinical research or case registry 5, 6

Essential consultations for mucosal involvement include ophthalmology, otolaryngology, urology/gynecology, and wound care services 2, 3
High-quality supportive care remains the priority regardless of specific immunomodulatory interventions chosen 1, 4