Suspected SJS/TEN
Patient with acute skin blistering/detachment after medication exposure
All Pathways
DermatologyEmergency
Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis (SJS/TEN)
Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis (SJS/TEN): Suspected SJS/TEN → STOP Culprit Drug Immediately → Assess Body Surface Area (BSA) Deta...
Pathway Overview
18 steps
Algorithm Steps
18 total
01Start 02Action STOP Culprit Drug Immediately
Identify and discontinue suspected causative medication
- Most common: Allopurinol, anticonvulsants (carbamazepine, phenytoin, lamotrigine)
- Sulfonamides, NSAIDs, antibiotics (fluoroquinolones)
- Onset typically 1-3 weeks after starting drug
- Stop ALL non-essential medications
03Decision Assess Body Surface Area (BSA) Detachment
Calculate percentage of skin with epidermal detachment
- Include: blistered areas + Nikolsky-positive areas
- Nikolsky sign: lateral pressure causes skin slippage
04Action SJS: <10% BSA
Stevens-Johnson Syndrome
- Mortality 1-5%
- May manage in dermatology ward
- Ophthalmology consult essential
05Action Calculate SCORTEN Score
Severity-of-illness score for prognosis (Day 1 & 3)
- Age >40 years (+1)
- Heart rate >120/min (+1)
- Cancer/hematologic malignancy (+1)
- BSA detachment >10% (+1)
- BUN >28 mg/dL (>10 mmol/L) (+1)
- Serum bicarbonate <20 mmol/L (+1)
- Serum glucose >252 mg/dL (>14 mmol/L) (+1)
06Action SCORTEN 0-1: Mortality 3%
Low risk - standard care
07Action Supportive Care (All Patients)
Burn-center level care principles
- Fluid resuscitation: 2-3 mL/kg/% BSA/day (less than burns)
- Temperature: Warm environment (30-32°C)
- Wound care: Non-adherent dressings, leave blisters intact if possible
- Nutrition: Early enteral feeding, high protein (1.5-2 g/kg/day)
- Pain: Adequate analgesia
- Electrolytes: Monitor and replace K+, Na+, Mg2+
- Infection surveillance: Cultures if fever, avoid prophylactic antibiotics
08Warning Ophthalmology Emergency
Ocular involvement in 50-90% of cases
- URGENT ophthalmology consult within 24h
- Preservative-free lubricants every 1-2 hours
- Topical corticosteroids per ophthalmology
- Symblepharon prevention with glass rod
- Amniotic membrane transplant may prevent scarring
09Action Ongoing Monitoring
Daily assessment until stable
- Serial SCORTEN (best predictive value Day 3)
- BSA progression vs re-epithelialization
- Infection surveillance
- Organ function (renal, hepatic, pulmonary)
- Fluid balance
- Nutritional status
10Outcome Re-epithelialization
Typically 2-4 weeks if surviving acute phase
- Long-term complications common
- Ophthalmology follow-up essential
- Allergy card/documentation
- Screen family for HLA-B*5801 (allopurinol), HLA-B*1502 (carbamazepine)
11Warning Death / Organ Failure
Sepsis and multi-organ failure main causes
- Overall mortality: SJS 1-5%, overlap 10-30%, TEN 30-50%
- Higher in elderly, comorbidities
- Early palliative care discussion if SCORTEN ≥4
12Decision Immunomodulatory Therapy
Consider specific treatment based on severity
13Action Cyclosporine (Preferred)
First-line immunomodulatory therapy
- Dose: 3-5 mg/kg/day in 2 divided doses
- Duration: 10-14 days, taper over 1 week
- IV if unable to swallow (1/3 of oral dose)
- Monitor: Renal function, BP, Mg2+
- Evidence: Reduced mortality (SMR 0.32)
- Path rejoins step 09Shared downstream outcome
14Action Alternative Therapies
If cyclosporine contraindicated
- IVIG: 0.5-1 g/kg/day x 3-4 days (controversial)
- Etanercept: 50mg SC x1 (emerging evidence)
- Corticosteroids: Short pulse may reduce progression
- IVIG + Corticosteroids: May be synergistic
- Avoid: Prolonged high-dose steroids alone
- Path rejoins step 09Shared downstream outcome
15Action SCORTEN 2-3: Mortality 12-35%
Moderate risk - intensive monitoring
- Path rejoins step 07Shared downstream outcome
16Warning SCORTEN ≥4: Mortality >58%
High risk - ICU/palliative discussion
- Path rejoins step 07Shared downstream outcome
17Action SJS/TEN Overlap: 10-30% BSA
Intermediate severity
- Mortality 10-30%
- Burn unit admission recommended
- Multi-organ monitoring
- Path rejoins step 05Shared downstream outcome
18Warning TEN: >30% BSA
Toxic Epidermal Necrolysis - Life-threatening
- Mortality 30-50%
- MANDATORY burn unit/ICU admission
- Aggressive resuscitation required
- Path rejoins step 05Shared downstream outcome
Guideline Source
S3 Guideline: Diagnosis and Treatment of Epidermal Necrolysis
Clinical Safety Information
Clinical Decision Support — Not a Substitute for Clinical Judgment
Individual patient factors may require deviation from these recommendations.
Known Limitations
- Does not replace burn unit/dermatology specialist consultation
- Drug dosing requires individual patient assessment and renal function
- Immunomodulatory therapy choice may vary by institution
- Pediatric dosing may differ - consult pediatric guidelines
- Does not address long-term sequelae management
Contraindicated Populations
pediatric_requires_adjustment
Applicable Regions
EUUSglobal
EU: Based on German S3 Guideline 2024
US: Consider institutional burn unit protocols
Version 1Next review: 2028-01-01
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Related Resources
Frequently Asked Questions
What is the Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis (SJS/TEN)?
The Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis (SJS/TEN) is a emergency clinical algorithm for Dermatology. It provides a structured decision tree to guide clinical decision-making, based on S3 Guideline: Diagnosis and Treatment of Epidermal Necrolysis.
What guideline is the Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis (SJS/TEN) based on?
This algorithm is based on S3 Guideline: Diagnosis and Treatment of Epidermal Necrolysis (DOI: 10.1111/ddg.15515).
What are the limitations of the Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis (SJS/TEN)?
Known limitations include: Does not replace burn unit/dermatology specialist consultation; Drug dosing requires individual patient assessment and renal function; Immunomodulatory therapy choice may vary by institution; Pediatric dosing may differ - consult pediatric guidelines; Does not address long-term sequelae management. Individual patient factors may require deviation from these recommendations.
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