What is the treatment for Stevens-Johnson syndrome (SJS) caused by lamotrigine (Lamictal)?

Treatment of Lamotrigine-Induced Stevens-Johnson Syndrome

Immediately discontinue lamotrigine and transfer the patient to a burn center or intensive care unit with dermatology consultation for comprehensive supportive care, as this is the cornerstone of management that directly impacts mortality. 1

Immediate Actions

Drug Discontinuation and Assessment

• Stop lamotrigine immediately upon suspicion of SJS, as lamotrigine is one of the most notorious causative agents for this life-threatening reaction 1, 2
• Document all medications taken in the prior 2 months, including over-the-counter preparations 1
• Calculate SCORTEN within the first 24 hours of admission to predict mortality risk (uses 7 clinical parameters including age, BSA involvement, heart rate, serum glucose, bicarbonate, BUN, and presence of malignancy) 1

Transfer to Specialized Care

• Patients with >10% body surface area (BSA) epidermal loss must be transferred without delay to a burn center or ICU experienced in managing extensive skin loss 1
• Rapid admission to specialized centers is associated with improved survival, while delayed transfer increases mortality 1
• Establish a multidisciplinary team coordinated by dermatology and/or plastic surgery, including intensive care, ophthalmology, and specialist nursing 1

Supportive Care Management

Fluid Resuscitation

• Initiate fluid replacement using the formula: body weight (kg) × % BSA epidermal detachment = mL/hour 1
• This is significantly less aggressive than burn formulas (Parkland); overaggressive resuscitation causes pulmonary, cutaneous, and intestinal edema 1
• Monitor for end-organ hypoperfusion while avoiding fluid overload 1

Wound Care

• Cleanse wounds regularly with warmed sterile water, saline, or chlorhexidine (1:5000) 1
• Apply greasy emollients (50% white soft paraffin with 50% liquid paraffin) over all skin, including denuded areas; consider aerosolized formulations to minimize shearing forces 1
• Use appropriate dressings on exposed dermis to reduce fluid/protein loss, limit microbial colonization, and control pain 1
• Avoid prophylactic systemic antibiotics; only institute antimicrobial therapy if clinical signs of infection develop (confusion, hypotension, reduced urine output, oxygen desaturation, increased skin pain) 1

Nutritional Support

• Provide continuous enteral nutrition via oral route or nasogastric tube if oral intake is precluded by buccal mucositis 1
• Deliver 20-25 kcal/kg daily during the early catabolic phase 1
• Increase to 25-30 kcal/kg daily during the anabolic recovery phase 1

Pain Management

• Use the WHO analgesic ladder approach 1
• Provide adequate background simple analgesia for comfort at rest 1
• Add morphine-based opiate regimen for moderate-to-severe pain uncontrolled by simple analgesia, delivered enterally or via infusion 1
• Monitor level of consciousness, respiratory rate, and oxygen saturation carefully with opiate use 1

Additional Supportive Medications

• Low molecular weight heparin for prophylactic anticoagulation in immobile patients to prevent venous thromboembolism 1
• Proton pump inhibitor for gastric protection in patients who cannot establish enteral nutrition 1
• Consider recombinant human G-CSF in neutropenic patients to reduce infection risk and potentially enhance re-epithelialization 1

Systemic Immunomodulatory Therapy

Corticosteroid Therapy

• For Grade 3-4 SJS/TEN, first-line systemic treatment is IV methylprednisolone 0.5-1 mg/kg for Grade 3, or 1-2 mg/kg for Grade 4, tapering when toxicity resolves 3, 4
• Corticosteroids have been used successfully in multiple case reports of lamotrigine-induced SJS 2, 4, 5, 6

IVIG Consideration

• High-dose IVIG (2-3 g/kg over 3-5 days, typically 1 g/kg/day for 3 days) may be added in severe or steroid-unresponsive cases 3
• Consider IVIG for: severe disease (Grade 4) with >10% BSA involvement, steroid-unresponsive cases, rapidly progressive disease, or pediatric patients 3
• Note that UK guidelines state insufficient evidence to recommend IVIG routinely and suggest it should only be administered under specialist supervision in clinical research or case registry contexts 3
• Meta-analysis shows no overall survival benefit compared to supportive care alone (OR 1.00,95% CI 0.58-1.75), though high-dose regimens may be superior to low-dose 3
• Monitor for thromboembolic events, renal dysfunction, and aseptic meningitis during IVIG treatment 3

Ophthalmologic Management

• 74% of SJS/TEN patients develop acute ocular involvement (conjunctivitis, pseudomembrane formation, corneal/conjunctival epithelial defects) 1
• Obtain ophthalmology consultation immediately, as 50-63% of patients with acute involvement develop chronic complications (severe dry eyes, trichiasis) 1
• Neither severity of systemic disease nor grade of acute ocular disease predicts late complications; all patients require long-term ophthalmologic follow-up 1

Critical Pitfalls to Avoid

• Do not delay transfer to specialized care - this is associated with increased mortality 1
• Avoid polytherapy with antiepileptic drugs when possible, as this increases adverse effect frequency 4
• Do not use prophylactic antibiotics indiscriminately - this increases Candida colonization and promotes resistant organisms 1
• Do not underestimate fluid requirements but also avoid overaggressive resuscitation using burn formulas 1
• Recognize that lamotrigine-induced SJS can occur despite appropriate dosing and gradual titration 5

Prognosis and Monitoring

• SCORTEN score predicts mortality: score of 0-1 = 3.2% mortality, score of 5+ = 90% mortality 1
• Re-epithelialization may occur within days or take weeks to complete 1
• Most patients show substantial improvement within 3-7 days with appropriate management 5, 6
• Residual post-inflammatory hyperpigmentation is common 2