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

Management of SJS/TEN with SCORTEN >3

Patients with SJS/TEN and SCORTEN >3 require immediate transfer without delay to a burn center or specialized ICU, as they face predicted mortality ranging from 32% to 99% and need intensive multidisciplinary supportive care focused on preventing sepsis, managing extensive skin loss, and providing aggressive organ support. 1, 2

Immediate Transfer and Care Setting

Transfer to a specialized center is the single most critical intervention—delayed transfer significantly increases mortality. 1, 2

• Admit patients with >10% BSA epidermal loss (which typically corresponds to SCORTEN >3) to a burn center or ICU with specific SJS/TEN experience immediately 1
• Rapid admission to specialized centers has been demonstrated to improve survival in multiple studies and systematic reviews 1, 2
• Barrier-nurse in a side room with controlled humidity, pressure-relieving mattress, and ambient temperature raised to 25-28°C 1

Multidisciplinary Team Assembly

The SJS/TEN MDT must be coordinated by a specialist in skin failure (dermatology/plastic surgery) and include: 1

• Intensive care physicians
• Ophthalmology (mandatory within 24 hours) 2
• Specialist skincare nursing
• Additional input from: respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, pharmacy 1

Prognostic Context for SCORTEN >3

Understanding the mortality risk guides intensity of care: 1, 2

• 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

Initial Assessment Within 24 Hours

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

Critical Supportive Care Measures

Fluid Management

• Establish peripheral venous access through non-lesional skin when possible, changing peripheral lines every 48 hours 2
• Monitor fluid balance meticulously with urinary catheterization if urogenital involvement causes dysuria/retention 2
• Critical balance: adequate resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload that causes pulmonary, cutaneous, and intestinal edema 2

Respiratory Management

• Respiratory symptoms and hypoxemia on admission require urgent intensivist discussion and rapid ICU transfer, as deterioration requiring mechanical ventilation is likely 1
• Fibreoptic bronchoscopy should be undertaken to identify bronchial involvement, evaluate prognosis, and investigate pneumonitis by bacterial sampling 1

Skin Management Protocol

Minimize shearing forces during all patient handling and positioning—this is absolutely critical as lesional epidermis detaches easily with any friction. 1, 2

Wound Care Approach

• Day-to-day bedside care must be undertaken by specialist nurses familiar with skin fragility disorders 1
• Warn all attending clinicians unfamiliar with epidermal detachment problems before they examine the patient 1
• Cleanse wounds by gentle irrigation with warmed sterile water, saline, or chlorhexidine (1:5000) 2
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis including denuded areas 2
• Frequent application of bland emollient to whole skin supports barrier function, reduces transcutaneous water loss, and encourages re-epithelialization 1

Blister and Dressing Management

• Leave detached epidermis in situ as biological dressing; decompress blisters by piercing and expressing fluid 2
• Apply non-adherent dressings (Mepitel or Telfa) to denuded dermis with secondary foam dressing to collect exudate 2
• Use appropriate dressing on exposed dermis to reduce fluid and protein loss, limit microbial colonization, help pain control, and accelerate re-epithelialization 1
• Apply topical antimicrobials to sloughy areas only (consider silver-containing products), guided by local microbiology 2

Surgical Approach Consideration

A surgical approach involves debridement of detached epidermis followed by physiological wound closure using biosynthetic xenograft or allograft, and can be considered following: 1

• Failure of conservative management
• Clinical deterioration
• Extension of epidermal detachment
• Local sepsis/subepidermal pus
• Delayed healing
• Wound conversion (spontaneous progression of superficial skin loss into deeper cutaneous defect)

Infection Surveillance and Management

CRITICAL PITFALL: Do NOT use prophylactic systemic antibiotics—this increases resistant organism colonization without benefit. 1, 2

Monitoring Protocol

• Take swabs for bacterial and candidal culture from three lesional areas on alternate days throughout acute phase 2
• Denuded dermis becomes coated with necrotic debris and acts as substrate for microbial colonization, initially by Staphylococcus aureus and later by Gram-negative rods (especially Pseudomonas aeruginosa) 1
• Monitor carefully for clinical signs of infection: confusion, hypotension, reduced urine output, decreased oxygen saturation, increased skin pain, rising C-reactive protein 2

Antibiotic Use

• Administer systemic antibiotics ONLY when clinical infection is documented 2
• Cutaneous infection impairs re-epithelialization and may lead to systemic sepsis, which is the most common cause of death in SJS/TEN 1

Ophthalmologic Management

Ophthalmology consultation within 24 hours is mandatory with daily examinations throughout the acute phase—delayed involvement leads to permanent ocular sequelae. 2

Daily Ocular Care Regimen

• Two-hourly application of preservative-free lubricant (hyaluronate or carmellose drops) 2
• 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) 2
• Never perform blind sweeping of fornices with cotton buds or glass rods—this causes damage 2
• Topical antibiotics when corneal fluorescein staining or ulceration present 2
• Topical corticosteroids under ophthalmologist supervision may reduce ocular surface damage 2

Pharmacologic Interventions

While no active therapeutic regimen with unequivocal benefit exists for SJS/TEN, and only one randomized controlled trial (thalidomide) was discontinued prematurely due to excess deaths, several agents show promise: 1

Cyclosporine

• 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

Intravenous Immunoglobulin (IVIg)

The evidence for IVIg is mixed: 1

• Overall mortality rate of 221 patients with TEN and SJS-TEN overlap treated with IVIg was 19.9%
• Pooled odds ratio for mortality comparing IVIg and supportive care was 1.00 (95% CI 0.58-1.75; P = 0.99), showing no clear benefit
• Pediatric patients treated with IVIg had significantly lower mortality than adults (0% vs 21.6%; P = 0.01)
• These findings should be interpreted with caution given major methodological limitations

Critical Pitfalls to Avoid

1. Delayed transfer to specialized center—significantly increases mortality 1, 2
2. Prophylactic antibiotics—increases resistant organism colonization without benefit 1, 2
3. Aggressive fluid overload—causes pulmonary and tissue edema 2
4. Rough skin handling—causes further epidermal detachment 1, 2
5. Delayed ophthalmology involvement—leads to permanent ocular sequelae 2
6. Continuing culprit medication—worsens condition and increases mortality 2
7. Blind sweeping of ocular fornices—causes additional ocular damage 2