How should Stevens-Johnson syndrome be managed and treated?

Stevens-Johnson Syndrome Treatment

Immediately stop the suspected culprit drug, transfer patients with >10% body surface area (BSA) epidermal loss to a burn center or ICU experienced in SJS/TEN management, and prioritize high-quality multidisciplinary supportive care over any specific immunomodulatory therapy. 1

Immediate Actions

Drug Withdrawal

• Stop all suspected medications immediately - this is the single most critical intervention affecting mortality 1
• Document all medications taken in the 2 months prior to symptom onset
• List common culprits: allopurinol, carbamazepine, lamotrigine, nevirapine, phenytoin, sulfamethoxazole, NSAIDs 1

Severity Assessment & Transfer

• Calculate SCORTEN within 24 hours of admission to predict mortality (ranges 1-99% based on 7 parameters) 1
• Transfer to burn center or specialized ICU if >10% BSA involvement - delayed transfer increases mortality 1
• Rapid admission to burn centers demonstrates improved survival in multiple studies 1

Supportive Care (The Cornerstone of Treatment)

Environment & Nursing

• Barrier-nurse in side room with controlled humidity 1
• Ambient temperature 25-28°C 1
• Pressure-relieving mattress 1
• Minimize shearing forces - careful handling essential as epidermis detaches easily 1
• Specialist nurses familiar with skin fragility disorders should provide bedside care 1

Skin Management

Conservative approach (first-line):

• Irrigate wounds gently with warmed sterile water/saline or chlorhexidine (1:5000) 1
• Apply greasy emollient (50% white soft paraffin + 50% liquid paraffin) over entire epidermis including denuded areas - consider aerosolized formulations 1
• Leave detached epidermis in situ as biological dressing - decompress blisters by piercing and fluid expression 2, 1
• Apply topical antimicrobials to sloughy areas only (consider silver-containing products, but limit if extensive areas due to absorption risk) 1

Surgical approach (if conservative fails):

• Consider debridement under general anesthesia if clinical deterioration, extending detachment, local sepsis, delayed healing, or wound conversion occurs 1
• Apply biosynthetic xenograft or allograft after debridement 2, 1
• Should only be performed in experienced pediatric burn centers 2

Pain Management

• Use validated pain assessment tools daily in conscious patients 2
• Administer IV opioid infusions for those not tolerating oral medication 2
• Patient-controlled analgesia where appropriate with acute pain team involvement 2
• Consider sedation/general anesthesia for dressing changes and patient handling 2
• May keep patient sedated and ventilated in ICU for duration of acute phase 2

Infection Prevention

Critical caveat: Systemic sepsis is the most common cause of death in SJS/TEN 1

• Do NOT give prophylactic systemic antibiotics - increases colonization with Candida albicans 1
• Only institute antimicrobials if clinical signs of infection present 1
• Monitor for sepsis signs: confusion, hypotension, reduced urine output, decreased oxygen saturation, increased skin pain 1
• Watch for monoculture on swabs (previously mixed growth) indicating predominant invasive organism 1
• Take viral swabs from eroded areas if HSV suspected 2

Fluid Management

• Use modified formula: body weight/% BSA epidermal detachment for first 3 days 1
• Requirements are LOWER than Parkland formula for burns 1
• Avoid overaggressive resuscitation - causes pulmonary, cutaneous, and intestinal edema 1

Multidisciplinary Team Coordination

Core team must include: 1

• Dermatology and/or plastic surgery (coordinator)
• Intensive care
• Ophthalmology
• Specialist skincare nursing

Additional specialists as needed:

• Respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, pharmacy 1

Mucosal Care

Ocular (Critical for Long-term Quality of Life)

• Urgent ophthalmology review on admission 2, 1
• Daily ophthalmology review during acute phase including: 2
  • Fluorescein staining to assess corneal/conjunctival epithelial loss
  • Removal of pseudomembranes
  • Breakdown of conjunctival adhesions
• Daily gentle saline irrigation to remove mucous/debris 2
• Broad-spectrum topical antibiotic prophylaxis (e.g., moxifloxacin drops 4x daily) if corneal staining or ulceration 1
• Prevent corneal exposure in unconscious patients - use plastic wrap with thin ointment layer or long-lasting ophthalmic ointment 2, 1

Oral

• Daily oral review during acute phase 2, 1
• White soft paraffin ointment to lips every 2 hours 2, 1
• Warm saline mouthwashes or oral sponge cleaning daily 2, 1
• Benzydamine hydrochloride spray every 2-4 hours, especially before eating 2, 1
• Potent topical corticosteroid mouthwash (betamethasone sodium phosphate) 4x daily 2, 1
• Consider clobetasol propionate 0.05% cream/ointment in infants 2

Urogenital

• Daily urogenital review 1
• White soft paraffin ointment every 4 hours 1
• Potent topical corticosteroid ointment once daily to involved, non-eroded surfaces 1
• Silicone dressing (e.g., Mepitel) to eroded areas 1

Immunomodulatory Therapy (Controversial)

There is NO conclusive evidence demonstrating benefit of any immunomodulatory intervention over high-quality supportive care alone. 1

The evidence for corticosteroids, IVIg, cyclosporine, and TNF-inhibitors is of insufficient quality with major ascertainment bias, low patient numbers, variation in timing/intervention, and inconsistent results 1. Even among experienced clinicians, there is lack of consensus 1.

If active therapy is considered:

• Should be given under supervision of specialist skin failure MDT 1
• Ideally in context of clinical research or case registry 1
• Recent reviews (2025) confirm ongoing controversy with no standardized approach 3, 4, 5

Discharge Planning & Follow-up

• Provide written information about drugs to avoid 1
• Encourage MedicAlert bracelet 1
• Document drug allergy in patient notes and inform all involved physicians 1
• Report to national pharmacovigilance authorities 1
• Arrange dermatology outpatient appointment within weeks of discharge 1
• Ophthalmology follow-up if ocular involvement 1
• Mental health support is essential - survivors experience anxiety, depression, flashbacks, medical distrust, and social isolation 6
• Coordinate postdischarge care BEFORE discharge - patients report feeling abandoned without adequate support or physician knowledge about long-term sequelae 6

Long-term Sequelae

Most frequent complications are cutaneous, ocular, and psychological 3. Survivors experience ongoing skin issues, visual impairment (potentially blindness), lack of functional autonomy, career impacts, and significant psychological distress 6. Preemptive discharge education and SJS/TEN-specific care coordination are critically lacking in current practice 6.