What is the management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TENS) with a SCORTEN (Severity-of-illness score for Toxic Epidermal Necrolysis) score of more than 3?

Management of SJS/TEN with SCORTEN Score >3

Patients with SJS/TEN and SCORTEN >3 require immediate transfer to a burn center or specialized ICU, as they face a predicted mortality of 32-99% and need intensive multidisciplinary care with aggressive supportive measures to prevent fatal complications, particularly sepsis. 1

Immediate Care Setting and Team Assembly

Transfer without delay to a burn center or ICU with SJS/TEN experience is mandatory for patients with >10% BSA epidermal loss, which typically corresponds to SCORTEN >3. 1 Rapid admission to specialized centers has been demonstrated to improve survival, while delayed transfer increases mortality. 1

Multidisciplinary Team Requirements

The care team must be coordinated by a specialist in skin failure (dermatology or plastic surgery) and include: 1

• Intensive care physicians for hemodynamic management
• Ophthalmology for daily ocular assessments
• Specialist skincare nursing familiar with fragile skin handling
• Additional consultants as needed: respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, and pharmacy 1

Environmental Controls

Barrier-nurse the patient in a side room with: 1

• Ambient temperature raised to 25-28°C to prevent hypothermia from thermoregulatory dysfunction
• Humidity control
• Pressure-relieving mattress to minimize shearing forces on fragile skin

Comprehensive Initial Assessment

Within the first 24 hours, perform: 1

• Detailed medication history covering 2 months prior to symptom onset, including all over-the-counter preparations 1
• Full physical examination with baseline body weight, vital signs, and oxygen saturation 1
• Laboratory investigations: FBC, U&E, LFT, glucose, magnesium, phosphate, bicarbonate, mycoplasma serology 1
• Chest X-ray to assess pulmonary involvement 1
• Skin biopsy for histological confirmation 1
• SCORTEN calculation to predict mortality and guide intensity of care 1

Critical Supportive Care Measures

Fluid and Hemodynamic Management

Establish peripheral venous access through non-lesional skin when possible, changing peripheral lines every 48 hours. 1 Monitor fluid balance meticulously with urinary catheterization if urogenital involvement causes dysuria/retention or for accurate output monitoring. 1 The goal is adequate resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload that causes pulmonary, cutaneous, and intestinal edema. 2

Skin Management Protocol

Conservative approach (initial for all patients): 1

• Minimize shearing forces during all patient handling and positioning—this is critical as lesional epidermis detaches easily 1
• Cleanse wounds by gentle irrigation with warmed sterile water, saline, or chlorhexidine (1:5000) 1, 2
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis including denuded areas 1
• Leave detached epidermis in situ as biological dressing; decompress blisters by piercing and expressing fluid 1, 2
• Apply non-adherent dressings (Mepitel or Telfa) to denuded dermis with secondary foam dressing to collect exudate 1
• Topical antimicrobials to sloughy areas only (consider silver-containing products), guided by local microbiology 1

Surgical approach (if conservative management fails): 1

Consider transfer to burn center for surgical debridement if there is: 1

• Clinical deterioration
• Extension of epidermal detachment
• Subepidermal pus or local sepsis
• Delayed healing or wound conversion

Surgical management involves debridement under general anesthesia with topical antimicrobial cleansing (betadine or chlorhexidine), possible Versajet debridement, and physiological closure with Biobrane/allograft/xenograft for non-infected confluent areas. 1

Infection Surveillance and Management

Critical pitfall to avoid: Do NOT use prophylactic systemic antibiotics. 1 Indiscriminate antibiotic use increases skin colonization, particularly with Candida albicans and resistant organisms. 1, 2

Instead: 1

• Take swabs for bacterial and candidal culture from three lesional areas on alternate days throughout acute phase
• Monitor carefully for clinical signs of infection: confusion, hypotension, reduced urine output, decreased oxygen saturation, increased skin pain, rising C-reactive protein 1
• Administer systemic antibiotics ONLY when clinical infection is documented 1

Sepsis is the most common cause of death in SJS/TEN, with initial colonization by Staphylococcus aureus followed by Gram-negative rods (especially Pseudomonas aeruginosa). 1

Nutritional Support

Insert nasogastric tube if oral intake is precluded by buccal mucositis. 1 Provide continuous enteral nutrition: 2

• 20-25 kcal/kg daily during catabolic phase
• 25-30 kcal/kg during recovery phase

Pain Management

Provide adequate background simple analgesia with additional opioid analgesia for breakthrough pain. 2 The extensive denuded dermis causes severe pain requiring aggressive management.

Mucosal Care Protocols

Ophthalmologic Management

Ophthalmology consultation within 24 hours is mandatory with daily examinations throughout the acute phase. 1, 2 This is non-negotiable as late ocular complications occur in >50% of patients regardless of acute severity. 1

Daily regimen: 1, 2

• Two-hourly application of preservative-free lubricant (hyaluronate or carmellose drops) 1
• Daily ocular hygiene by ophthalmologist or trained nurse using saline irrigation, squint hook, and forceps to remove inflammatory debris and break down conjunctival adhesions (apply topical anesthetic first) 1, 2
• Never perform blind sweeping of fornices with cotton buds or glass rods—this causes damage 1
• Topical antibiotics when corneal fluorescein staining or ulceration present 1, 2
• Topical corticosteroids under ophthalmologist supervision may reduce ocular surface damage 1, 2
• Moisture chamber for unconscious patients to prevent corneal exposure, ulceration, and infection 1

Oral Care

• Anti-inflammatory oral rinse (benzydamine hydrochloride) every 3 hours, particularly before eating 2
• Antiseptic oral rinse twice daily 2
• Topical anesthetics (viscous lidocaine 2% or cocaine mouthwashes 2-5%) for severe oral discomfort 2
• Topical corticosteroids applied to oral mucosa 2
• Monitor for secondary infections; treat with appropriate antifungals or antivirals if HSV suspected 2

Urogenital Care

• Urinary catheterization when dysuria/retention occurs or for output monitoring 1, 2
• Regular examination of urogenital tract during acute illness 2
• White soft paraffin ointment applied to urogenital skin and mucosae every 4 hours 2
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 2

Pharmacologic Interventions

While supportive care is the cornerstone, consider: 2, 3, 4

• Cyclosporine (3 mg/kg daily for 10 days, tapered over 1 month) has shown benefit with reduced mortality in multiple studies 2
• Systemic corticosteroids (IV methylprednisolone pulse therapy) may be beneficial if started within 72 hours of onset 2

The evidence for specific immunomodulatory therapy remains mixed, but cyclosporine has the most consistent supportive data. 2, 4

Prognosis Context for SCORTEN >3

Understanding the mortality risk helps frame the intensity of intervention: 1

• SCORTEN 3: 32% predicted mortality
• SCORTEN 4: 62% predicted mortality
• SCORTEN 5: 85% predicted mortality
• SCORTEN 6: 95% predicted mortality
• SCORTEN 7: 99% predicted mortality

Critical Pitfalls to Avoid

• Delayed transfer to specialized center—this significantly increases mortality 1
• Prophylactic antibiotics—increases resistant organism colonization 1, 2
• Aggressive fluid overload—causes pulmonary and tissue edema 2
• Rough skin handling—causes further epidermal detachment 1
• Delayed ophthalmology involvement—leads to permanent ocular sequelae 1, 2
• Continuing culprit medication—worsens condition and increases mortality 2

Discharge Planning

When patient stabilizes: 2

• Provide written information about culprit drug(s) and cross-reactive medications to avoid
• Encourage MedicAlert bracelet with culprit drug name
• Document drug allergy in all medical records
• Report adverse drug reaction to pharmacovigilance authorities
• Warn about potential fatigue/lethargy for weeks post-discharge
• Arrange long-term ophthalmology follow-up regardless of acute ocular severity
• Consider referral to support groups