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    Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Treatment

    Critical Care / Resuscitation, Ears, Eyes, Nose, and Throat, Inflammatory

    Last Updated Apr 21, 2021
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    By Richard Xiang, Jordanna Roesler, Gord McInnes, Julian Marsden

    Context

    • 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.

    Recommended Treatment

    • 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.

    Criteria For Hospital Admission

    • 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.

    Criteria For Transfer To Another Facility

    See above.

    Criteria For Close Observation And/or Consult

    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).

    Criteria For Safe Discharge Home

    • 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.

    Quality Of Evidence?

    Justification

    There are no clear treatment guidelines for SJS/TEN due to the lack of large controlled studies and infrequency of SJS/TEN.

    There are varying levels of evidence for the use of IVIG, with some studies claiming a benefit of IVIG administration during the early stage of SJS/TEN, in conjunction with IV corticosteroids.

    Reference: Aihara, M. (08/2015). Efficacy of additional i.v. immunoglobulin to steroid therapy in stevens-johnson syndrome and toxic epidermal necrolysis Japanese Dermatological Association. doi:10.1111/1346-8138.12925.

    Cyclosporine also requires more research on larger sample sizes. However, in a single centre retrospective review of 71 patients with TEN/SJS, patients who received cyclosporine had a relative mortality benefit compared to IVIG.

    Low

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