What is the treatment for drug-induced Stevens-Johnson syndrome (SJS)?

Treatment of Drug-Induced Stevens-Johnson Syndrome

Immediately discontinue the suspected culprit drug and transfer the patient to a specialized burn unit or ICU for multidisciplinary care, as this is the single most critical intervention that directly impacts survival. 1, 2, 3

Immediate Actions (First 24 Hours)

• Stop all suspected medications immediately upon first sign of rash or mucosal involvement, as delayed withdrawal significantly increases mortality risk 1, 2, 3
• Transfer to an age-appropriate specialist burn unit or ICU with dermatology expertise 1, 2, 3
• Calculate SCORTEN within the first 24 hours to predict mortality risk (scores range 0-7, with higher scores indicating greater mortality) 2, 3
• Document all medications taken in the previous 2 months, including over-the-counter products, with exact start dates 3

Common pitfall: Delayed transfer to specialized units significantly increases mortality—do not attempt outpatient management 3, 4

Diagnostic Workup

• Obtain complete blood count with differential, C-reactive protein, comprehensive metabolic panel (including liver function tests, renal function, electrolytes), coagulation studies, glucose, magnesium, phosphate, bicarbonate, and lactate 3
• Use the ALDEN algorithm to help define drug causality 2
• Perform skin biopsy showing full-thickness epidermal necrosis with minimal dermal inflammation 5, 4
• Test for potential infectious triggers, especially in children where infections cause up to 50% of cases 2

Systemic Immunomodulatory Therapy

For Grade 3-4 severity (>10% body surface area involvement):

• Administer IV methylprednisolone 0.5-1 mg/kg daily if initiated within 72 hours of onset, converting to oral corticosteroids upon response and tapering over at least 4 weeks 1, 3
• For immune checkpoint inhibitor-induced cases, use IV methylprednisolone 1-2 mg/kg given the T-cell immune-directed mechanism 1

Alternative/adjunctive therapy:

• Consider cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month, as it has demonstrated reduced mortality compared to predicted rates in multiple case series 1, 2, 3, 5
• IVIG may be considered in severe or steroid-unresponsive cases, though evidence is limited 1, 4

Important controversy: While corticosteroids may suppress progression, they increase infection risk—the leading cause of death. The guideline evidence supports their use when started early (<72 hours) with close monitoring for infection 1, 3, 4

Supportive Care Management

Wound care:

• Apply topical emollients, petrolatum-based products, or dimethicone to affected skin 1
• Consider biological dressings (porcine xenograft) to protect dermis from desiccation and promote rapid re-epithelialization 6
• Consult wound care services for specialized management 1

Nutritional support:

• Provide continuous enteral nutrition delivering 20-25 kcal/kg daily, either orally or via nasogastric feeding 3

Prophylaxis:

• Administer low molecular weight heparin for venous thromboembolism prophylaxis in immobile patients 3
• Provide proton pump inhibitor for stress ulcer prophylaxis if enteral nutrition cannot be established 3
• Do not use prophylactic antibiotics—only treat culture-proven infections 6, 4

Multidisciplinary Consultation

Mandatory consultations within 24 hours:

• Ophthalmology for all patients with eye involvement to prevent permanent visual impairment 1, 3
• Dermatology or burn/plastic surgery for wound management 1, 2, 3

Additional consultations as indicated:

• Otolaryngology, urology, or gynecology for mucosal involvement to prevent stricture formation 1
• Pain/palliative care for symptom management 1

Discharge Planning and Long-Term Management

Documentation and patient education:

• Provide written information about the culprit drug and all cross-reactive medications to avoid permanently 1, 3
• Encourage patient to wear MedicAlert bracelet bearing the name of the culprit drug 1, 2
• Document drug allergy in medical records and inform all healthcare providers, especially primary care 1
• Report to national pharmacovigilance authorities (Yellow Card Scheme in UK, MedWatch in US) 1

Follow-up appointments:

• Dermatology or burn/plastic surgery within weeks of discharge to monitor for skin, oral, urogenital, respiratory, and gastrointestinal complications 1, 3
• Ophthalmology within weeks if eye involvement occurred, to monitor for chronic ocular complications 1, 3
• Psychological evaluation and support 1

Drug Hypersensitivity Testing

Do not perform routine drug hypersensitivity testing following SJS/TEN 2

If testing is considered for severe cases:

• Refer to specialist center with expertise in drug allergy 2
• Patch testing has variable sensitivity (9-62% depending on drug and population) 1
• In vitro tests (lymphocyte transformation tests) may be considered but have limited validation 1
• Never perform oral provocation studies—risk of life-threatening reactions 1, 2

High-Risk Medications and Genetic Testing

Most commonly implicated drugs:

• Sulfonamides (including trimethoprim-sulfamethoxazole) 7, 5, 4
• Anticonvulsants (carbamazepine, phenytoin) 1, 8, 5, 4
• NSAIDs (especially oxicam type, diclofenac) 9, 5, 4
• Allopurinol 5, 4
• Antimalarials 4

Genetic screening considerations:

• For carbamazepine: Test for HLA-B*1502 in patients of Asian ancestry (>15% prevalence in Hong Kong, Thailand, Malaysia, Philippines; ~10% Taiwan; ~4% North China) before initiating therapy 8
• For carbamazepine: HLA-A*3101 testing may identify risk for hypersensitivity reactions in European, Korean, and Japanese populations (>15% prevalence in Japanese, Native American, Southern Indian populations) 8
• Do not use carbamazepine in HLA-B*1502 positive patients unless benefits clearly outweigh risks 8

Critical caveat: Many HLA-positive patients will never develop SJS, and reactions still occur in HLA-negative patients—genetic testing supplements but does not replace clinical vigilance 8