• The acute phase of SJS/TEN lasts 8-12 days, with persistent fever, mucous membrane involvement and epidermal sloughing, leaving large, raw painful areas of denuded skin.
• The role of the emergency physician in SJS/TEN treatment involves early detection, withdrawal of offending medications/agents, early consultation with relevant specialists, supportive care and/or ICU/burn unit referral.
• Sterile handling, pain control, and fluid management are simple but effective interventions that can be initiated early.
• Other complications that require monitoring/intervention include electrolyte imbalances, bleeding, evaporative heat loss, hypovolemic shock, insulin resistance, bacteremia from aureus and Pseudomonas, hypercatabolism, and pneumonia.
• SCORTEN is a clinical tool that can be used to assess prognosis in patients with SJS/TEN.

There are no definitive treatments recommended for SJS/TEN at this time, however, IVIG/Corticosteroids and Cyclosporine have weak evidence supporting their use.

• Identify any new drugs started within the last 5-56 days (ALDEN tool) and remove the offending agent if possible. Drugs started <5 days ago are unlikely to be the offending agent. Early withdrawal of medication decreases mortality and improves prognosis, especially if done before blister development.

Supportive Management

• • Pain control
    • Mild pain: non-opioid analgesia – Acetaminophen/Ibuprofen.
    • Moderate-severe pain: Hydromorphone PO/IV.
  • Wound care
    • Sterile technique and reverse-isolation precautions.
    • Avoid debridement of non-viable epidermis.
    • Topical antiseptics such as 0.5% silver nitrate or 0.05% chlorhexidine solution.
  • Nutritional support and fluid and electrolyte management as necessary.
    • Fluid requirements for SJS/TEN are ~⅔ – ¾ off that of burn patients, give ~2ml/kg X % BSA affected.
    • NG tube as needed.
    • High calorie requirements.
  • Increasing ambient room temperature to 30-32°C to prevent heat loss.
  • Prevent, monitor and treat infections/superinfections as sepsis is the leading cause of death in SJS/TEN.
    • Routine cultures from skin, blood, gastric tubes and catheters.
    • 5% silver nitrate and 0.05% chlorhexidine to paint affected skin areas.
  • Monitor and manage vulvovaginal symptoms. If symptomatic:
    • Complete pelvic exam.
    • Prophylactic vaginal molds.
    • Menstrual suppression during the acute phase.
    • Intravaginal corticosteroids and topical antifungal agents.
  • Ocular Care
    • Ophthalmologic consultation.
    • Saline rinse and ointment or artificial tears for lubrication.
    • Daily topical erythromycin drops
    • Conjunctival hyperemia: topical corticosteroids and broad-spectrum antibiotics.

Immunomodulating Treatment (Limited Evidence)

• There are no curative therapeutic interventions known at this time.
• IVIG with short-term, high-dose corticosteroids.
• IVIG 2-3g/kg over 2-3 days.
• Immunosuppressive drugs: Cyclosporine 3-5 mg/kg per day.

Consider

• Mechanical ventilation.
• Phosphate repletion.
• Systemic antibiotics.
• Topical lubricants/steroid drops for acute ocular involvement.

Treatments Not Recommended

• Prophylactic Antibiotics.
• Silver Sulfadiazine.
• Debridement in the Emergency Department without biologic dressings available.
• Systemic corticosteroids – Shown to increase morbidity/mortality through increased risk of sepsis, delayed re-epithelialization and increased protein catabolism.
• IVIG – Increases risk of thrombotic, renal, and hematologic complications, especially in elderly patients or those with cardiovascular and renal comorbidities.
• Thalidomide – TNF-alpha inhibitor, increases mortality.

• All patients suspected to have SJS/TEN should be admitted to the hospital.
• Admit to ICU or burn unit if SCORTEN ≥ 2, and/or extensive skin detachment or comorbidities.
• The SCORTEN score can be used to prognosticate the severity of disease.
• SCORTEN is calculated based on 7 independent and easily obtainable lab values and has been found to accurately predict mortality.
  • Score of 0 or 1 with slow disease progression and limited skin involvement can be treated on non-specialized wards.
  • Scores of 2 or higher with more skin involvement or skin detachment >30% of BSA should be treated in the ICU or burn unit and transported to a specialized tertiary centre accordingly.

                                                                                                          Total: /7

SCORTEN: Score of toxic epidermal necrolysis.

*Malignancy: includes evolving cancer and hematologic malignancies.

See above.

All suspected cases of SJS/TEN require close observation with consultation to dermatology and other relevant specialists depending on other organs involved (i.e. Gastroenterology, Ophthalmology).

• Ongoing wound care, monitoring for signs of infection (purulent discharge, fevers etc.), adequate pain management including oral care for mucocutaneous lesions are important discharge considerations.
• Patients should return to the ED/hospital if their condition worsens or they develop signs of infection.
• Family and patient education, risk communication, and avoidance of drugs implicated in SJS/TEN.
• Medical alert jewelry.
• Follow up with a family physician to document the resolution of the skin/oral lesions.
• Follow up with a dermatologist within 6 months.

Follow up with relevant specialists depending on organs affected.

1. What are Stevens–Johnson syndrome and toxic epidermal necrolysis? (DermNet NZ)

   
   

2. SCORTEN Scale (MD Calc)

   
   

1. Torres‐Navarro, I. (09/2020). Accuracy of SCORTEN to predict the prognosis of Stevens‐Johnson syndrome/toxic epidermal necrolysis: A systematic review and meta‐analysis Blackwell Publishing. doi:10.1111/jdv.16137.

   
   

2. Guégan, S., Bastuji-Garin, S., Poszepczynska-Guigné, E., Roujeau, J.-C., and Revuz, J. 2006. Performance of the SCORTEN during the first five days of hospitalization to predict the prognosis of epidermal necrolysis. Journal of investigative dermatology 126(2): 272-276.

   
   

3. Sassolas, Bruno & Mockenhaupt, Maja & Dunant, A & Liss, Y & Bork, K & Haustein, Uwe-Fritjof & Vieluf, D & Roujeau, Jean-Claude & Louet, H. (2010). ALDEN, an Algorithm for Assessment of Drug Causality in Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: Comparison With Case–Control Analysis. Clinical pharmacology and therapeutics. 88. 60-8. 10.1038/clpt.2009.252.

   
   

4. Shanbhag, S. S., Chodosh, J., Fathy, C., Goverman, J., Mitchell, C., & Saeed, H. N. (2020). Multidisciplinary care in Stevens-Johnson syndrome. Therapeutic advances in chronic disease, 11, 2040622319894469.

   
   

5. Shiga S, Cartotto R. What are the fluid requirements in toxic epidermal necrolysis? J Burn Care Res. 2010 Jan-Feb;31(1):100-4. doi: 10.1097/BCR.0b013e3181cb8cb8.