SNOMED CT Toxic epidermal necrolysis disorder term
SNOMED CT Stevens Johnson syndrome disorder term
Laboratory Tests
Baseline severity and organ dysfunction
Severity labs
Complete blood count for cytopenias and infection signal
Leukopenia as severity marker
Neutrophilia as infection or stress marker
Electrolytes and renal function for SCORTEN and resuscitation
Urea mmol per L for SCORTEN element
Sodium for free water loss
Potassium for arrhythmia risk
Liver enzymes for hepatic involvement
Drug injury signal
Sepsis signal
Glucose mmol per L for SCORTEN element
Hyperglycemia as stress marker
Albumin for nutritional risk and capillary leak
Hypoalbuminemia as severity marker
Infection evaluation
Infection workup based on physiology
Blood cultures for fever with hemodynamic instability
Two sets before antibiotics when feasible
Urinalysis and urine culture for urinary symptoms or Foley
Secondary infection risk with mucosal disease
Wound culture if purulence or malodor
Avoid routine colonization cultures as treatment trigger
Lactate mmol per L for shock physiology
Trend response to resuscitation
Treatment monitoring labs
Monitoring labs tied to therapies
Coagulation studies for bleeding risk and liver dysfunction
INR trend
Fibrinogen if DIC concern
Creatine kinase for rhabdomyolysis concern
Prolonged immobilization
Severe skin pain
Arterial blood gas for respiratory failure
PaO2 mmHg
PaCO2 mmHg
Pregnancy test where applicable
Medication selection impact
Diagnostic Tests
Scoring Systems
SCORTEN
Timing
Calculate within first 24 hours of admission
Repeat at 48 hours for updated prognosis
Elements
Age over 40 years
Malignancy
Heart rate over 120 per minute
Initial epidermal detachment over 10 percent body surface area
Serum urea over 10 mmol per L
Serum glucose over 14 mmol per L
Serum bicarbonate under 20 mmol per L
Use in care planning
Higher score correlates with higher mortality
Trigger for ICU or burn center escalation
MRI
MRI role
Generally not first line
No routine role in TEN diagnosis
Indications when needed
Neurologic complication evaluation
Osteomyelitis evaluation in chronic wounds
Limitations
Transport risk in unstable patients
Delays definitive care
CT
CT role
Not required for TEN diagnosis
Clinical diagnosis supported by biopsy
Indications based on complications
CT chest for severe respiratory symptoms
CT abdomen pelvis for severe abdominal pain or sepsis source concern
Contrast considerations
Acute kidney injury risk
Hydration strategy if contrast needed
Ultrasound (or US)
Ultrasound role
Point of care ultrasound for shock evaluation
IVC assessment for volume status
Cardiac function for cardiogenic shock exclusion
Lung ultrasound for respiratory failure
B lines for pulmonary edema
Consolidation for pneumonia
Vascular ultrasound for thrombosis evaluation
DVT risk in immobility and inflammation
Disposition
Level of care selection
Admission pathway
ICU indications
Hemodynamic instability
Rising oxygen requirement
Altered mental status
Significant metabolic derangement
Burn center indications
TEN greater than 30 percent body surface area detachment
SJS TEN overlap with rapid progression
Significant mucosal involvement
Need for specialized wound care
Step down indications
Stable hemodynamics
Limited detachment with close monitoring capability
Transfer and timing
Transfer planning
Early transfer before peak skin loss
Improved access to multidisciplinary care
Pre transfer stabilization
Adequate analgesia
Initial fluid resuscitation started
Airway risk addressed
Documentation for receiving center
Medication timeline and suspected culprit list
Body surface area estimate
SCORTEN elements
Treatment
Stop triggers and remove inciting agents
Offending agent discontinuation
High risk drug stop immediately
Allopurinol
Anticonvulsants
Sulfonamide antibiotics
Nevirapine
NSAIDs
Medication reconciliation and substitution
Avoid cross reactive anticonvulsants
Use non aromatic alternatives when appropriate
Supportive care and resuscitation
Supportive bundle
Fluid resuscitation strategy
Crystalloid guided by urine output and hemodynamics
Urine output goal adults 0.5 mL per kg per hour
Urine output goal pediatrics 1 mL per kg per hour
Electrolyte replacement plan
Sodium correction gradual
Potassium repletion with ECG monitoring
Temperature management
Warm ambient room
Forced air warming if hypothermic
Glycemic management
Avoid severe hyperglycemia
Avoid hypoglycemia
Wound care and skin failure management
Wound care principles
Handling and dressings
Non adherent dressings
Silicone contact layer options
Petrolatum gauze options
Aseptic technique
Hand hygiene strict
Barrier precautions
Debridement approach
Avoid aggressive debridement of viable epidermis
Remove clearly necrotic detached tissue per burn team protocol
Topical agents
Petrolatum based emollients
Reduce friction
Reduce pain with dressing changes
Avoid topical sulfonamides if sulfonamide culprit suspected
Pain control and symptom relief
Analgesia and sedation
Opioid based regimen
Hydromorphone IV 0.2 mg to 0.5 mg
Repeat every 10 to 15 minutes until controlled
Transition to scheduled dosing when stable
Fentanyl IV 25 mcg to 50 mcg
Repeat every 5 to 10 minutes for procedures
Caution in hypotension
Adjuncts
Acetaminophen PO or IV 15 mg per kg
Maximum 4 g per day adult
Avoid in severe hepatic injury
Gabapentin PO 100 mg to 300 mg
Renal dose adjustment
Sedation monitoring
Pruritus control if present
Cetirizine PO 10 mg daily
Sedation lower than diphenhydramine
Renal adjustment in severe CKD
Airway and pulmonary involvement
Airway plan
Early airway consultation triggers
Progressive oral swelling
Stridor
Hypoxemia despite oxygen
Intubation considerations
Anticipated difficult airway with friable mucosa
Video laryngoscopy preference
Smaller endotracheal tube consideration
Gentle technique to reduce mucosal trauma
Eye care
Ocular injury prevention
Ophthalmology led protocol
Daily exam in acute phase
Early lysis of adhesions if needed
Lubrication
Preservative free artificial tears hourly while awake
Reduce epithelial injury
Reduce synechiae risk
Lubricating ointment at bedtime
Reduce exposure keratopathy
Caution with blurred vision
Topical antibiotics if epithelial defect
Erythromycin ophthalmic ointment as typical option
Avoid neomycin sensitization
Follow ophthalmology direction
Genitourinary and mucosal care
Genital care
Barrier ointment
Petrolatum based
Reduce adhesion formation
Topical steroid if recommended by specialist
Low to medium potency on vulvar skin
Avoid infected appearing areas
Foley avoidance when possible
Reduce urethral trauma
Reduce infection risk
Nutrition and GI support
Nutrition strategy
Early enteral feeding when feasible
High calorie high protein targets
Dietitian involvement
Swallowing limitation support
Soft diet
Liquid supplements
Stress ulcer prophylaxis if ICU level and risk factors
Proton pump inhibitor choice individualized
C difficile risk consideration
Thromboprophylaxis
VTE prevention
Pharmacologic prophylaxis unless contraindicated
Enoxaparin SC 40 mg daily
Renal dose adjustment if eGFR low
Platelet monitoring if heparin exposure history
Unfractionated heparin SC 5000 units every 8 to 12 hours
Use if renal failure
Bleeding risk reassessment daily
Mechanical prophylaxis
Intermittent pneumatic compression
Use when bleeding risk high
Infection management and antibiotics
Infection strategy
No routine prophylactic systemic antibiotics
Colonization common without invasive infection
Reserve for proven or strongly suspected infection
Empiric antibiotics if sepsis physiology
Broad spectrum choice guided by local resistance
Cover skin and soft tissue pathogens
Cover gram negatives if hospital acquired risk
De escalation after cultures
Narrow by organism
Stop if no infection evidence
Immunomodulatory therapy
Disease modifying therapy selection
Specialist guided decision making
Dermatology and burn team alignment
Consider within early disease window
Systemic corticosteroids
Consider short course early in select patients
Methylprednisolone IV 1 mg per kg per day
Reassess daily for infection signal
Taper or stop if worsening sepsis concern
Avoid prolonged courses
Infection risk
Delayed wound healing risk
Cyclosporine
Option in early TEN in appropriate patients
Cyclosporine PO 3 mg per kg per day in divided doses
Typical duration 7 to 10 days
Renal function monitoring daily
Blood pressure monitoring
Etanercept
Option in select protocols
Etanercept SC 50 mg once
Repeat 50 mg at 24 to 72 hours per protocol
Infection screening consideration
IVIG
Consider when used in local protocol
IVIG 1 g per kg per day
Duration 2 to 3 days
Thrombosis risk monitoring
Renal risk monitoring
Evidence grading documentation
Class IIb recommendation for immunomodulators in many pathways
ACEP Level C style consensus for ED initiation decisions
Special Populations
Pregnancy
Pregnancy considerations
Maternal fetal risk
Hypovolemia impacts uteroplacental perfusion
Sepsis increases fetal compromise risk
Medication selection
Avoid teratogenic substitutions
Coordinate with obstetrics and pharmacy
Fetal assessment
Gestational age based monitoring plan
Continuous monitoring if viable gestation and maternal instability
Geriatric
Older adult considerations
Higher baseline SCORTEN contribution
Age element increases risk estimate
Lower physiologic reserve
Polypharmacy complexity
Multiple candidate culprits
Drug drug interaction risk with cyclosporine
Delirium risk
Minimize sedating antihistamines
Frequent reorientation strategies
Pediatrics
Pediatric considerations
Etiology differences
Infection associated SJS more common than in adults
Medication triggers still important
Fluids and electrolytes
Higher maintenance needs per kg
Hypoglycemia risk
Weight based medication dosing
Opioid dosing per kg with monitoring
Immunomodulator dosing specialist directed
Background
Epidemiology
Epidemiology
Incidence rarity
TEN uncommon condition
Higher incidence with high risk drugs and immunosuppression
Mortality burden
Mortality increases with higher SCORTEN
Sepsis and respiratory failure common causes
Long term morbidity
Ocular sequelae
Genital scarring
Chronic skin dyspigmentation
Pathophysiology
Mechanism
Severe cutaneous adverse reaction
Cytotoxic T cell mediated keratinocyte apoptosis
Widespread epidermal necrosis
Barrier failure
Fluid loss and electrolyte derangement
Infection susceptibility
Thermoregulation loss
Mucosal injury
Epithelial sloughing
Adhesion formation risk
Therapeutic Considerations
Treatment rationale
Early culprit drug withdrawal
Strongest modifiable factor
Earlier stop correlates with better outcomes
Burn style supportive care
Proven benefit in skin failure states
Focus on fluids, wound care, nutrition, infection vigilance
Immunomodulation uncertainty
Mixed evidence across agents
Balance infection risk with potential disease arrest
Guideline style evidence language
ACEP Level C style consensus for early ED priorities
Class I recommendation for immediate culprit drug discontinuation in most pathways
Class IIb recommendation for selected immunomodulators based on institutional protocol
Patient Discharge Instructions
copy discharge instructions
Discharge only when appropriate
This condition usually requires admission
Discharge applies to resolved mild cases or post discharge follow up planning
Skin care at home
Gentle cleansing
Non adherent dressings as instructed
Emollient use as instructed
Eye care at home if involved
Lubrication schedule as directed by ophthalmology
Avoid contact lenses until cleared
Medication safety
Permanent avoidance of culprit medication
Avoid related drugs when advised
Medical alert identification
Follow up
Dermatology follow up within 1 week
Ophthalmology follow up within 48 to 72 hours if eye involvement
Primary care follow up for medication list reconciliation
Return now warning signs
New or worsening skin pain
New blisters or skin peeling
Fever
Mouth sores limiting fluids
Eye pain or vision change
Trouble breathing
Dizziness or fainting
Decreased urine output
References
Clinical guidelines and consensus
Guideline and consensus sources
British Association of Dermatologists guidance for SJS and TEN
Supportive care emphasis
Early multidisciplinary management
European dermatology and burn care consensus statements
Transfer to specialized centers
Ocular care early
UpToDate topic reviews for SJS and TEN
Diagnostic criteria and management overview
Immunomodulator summaries
Key studies and tools
Evidence based sources
SCORTEN original validation studies
Mortality prediction utility
Use within first 24 to 48 hours
Observational studies on culprit drug withdrawal timing
Earlier discontinuation associated with improved outcomes
Supports Class I style recommendation for drug stop
Comparative studies and meta analyses of cyclosporine
Signal for reduced mortality in some analyses
Renal and infection risk considerations
Trials and observational data for etanercept
Time to re epithelialization outcomes in some studies
Infection vigilance required
Systematic reviews of IVIG and corticosteroids
Mixed results across cohorts
Emphasis on protocolized and early use when chosen
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