Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis (SJS/TEN)

Algorithm Steps

1. ▶

   Start

   Suspected SJS/TEN

   Patient with acute skin blistering/detachment after medication exposure

   1. ●

      Action

      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
      1. ◆

         Decision

         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
         1. ●

            Action

            SJS: <10% BSA

            Stevens-Johnson Syndrome

            • Mortality 1-5%
            • May manage in dermatology ward
            • Ophthalmology consult essential
            1. ●

               Action

               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)
               1. ●

                  Action

                  SCORTEN 0-1: Mortality 3%

                  Low risk - standard care

                  1. ●

                     Action

                     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
                     1. ⚠

                        Warning

                        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
                        1. ●

                           Action

                           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
                           1. ✓

                              Outcome

                              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)
                           2. ⚠

                              Warning

                              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
                     2. ◆

                        Decision

                        Immunomodulatory Therapy

                        Consider specific treatment based on severity

                        1. ●

                           Action

                           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)
                        2. ●

                           Action

                           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
               2. ●

                  Action

                  SCORTEN 2-3: Mortality 12-35%

                  Moderate risk - intensive monitoring

               3. ⚠

                  Warning

                  SCORTEN ≥4: Mortality >58%

                  High risk - ICU/palliative discussion

         2. ●

            Action

            SJS/TEN Overlap: 10-30% BSA

            Intermediate severity

            • Mortality 10-30%
            • Burn unit admission recommended
            • Multi-organ monitoring
         3. ⚠

            Warning

            TEN: >30% BSA

            Toxic Epidermal Necrolysis - Life-threatening

            • Mortality 30-50%
            • MANDATORY burn unit/ICU admission
            • Aggressive resuscitation required