What is the treatment for a patient diagnosed with Stevens-Johnson Syndrome (SJS) or Toxic Epidermal Necrolysis (TEN)?

Treatment of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Immediate Life-Saving Actions

Immediately discontinue all suspected culprit medications and transfer patients with >10% body surface area (BSA) epidermal detachment to a specialized burn center or ICU with experience managing SJS/TEN within hours of diagnosis. 1 Delayed transfer significantly increases mortality risk. 2

Critical First Steps

• Stop the culprit drug immediately - this is the single most important intervention that directly impacts survival 1, 3
• Calculate SCORTEN within the first 24 hours to predict mortality (scores 0-7, with mortality ranging from 1% to 99%) 1, 2
• Document all medications taken in the previous 2 months, including over-the-counter products, with exact start dates 2, 3
• Transfer patients with >10% BSA involvement without delay to centers with multidisciplinary teams 1, 4

Specialized Care Environment

Patients must be barrier-nursed in a temperature-controlled side room (25-28°C) on a pressure-relieving mattress with humidity control. 1 This prevents hypothermia from extensive skin loss and reduces infection risk. 1

Supportive Care Framework

Fluid Management

• Establish adequate intravenous fluid replacement guided by urine output and hemodynamic parameters 1, 2
• Avoid overaggressive fluid resuscitation - this causes pulmonary, cutaneous, and intestinal edema 2, 4
• Monitor fluid balance carefully with urinary catheterization when indicated 1, 2
• Progressively increase oral fluids as mouth involvement improves 1

Wound Care Protocol

• Handle skin with extreme care to minimize shearing forces that cause further epidermal detachment 1, 2, 4
• Irrigate wounds gently using warmed sterile water, saline, or chlorhexidine (1:5000) 1, 4
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis including denuded areas every few hours 1, 2, 4
• Leave detached epidermis in situ to act as biological dressing - decompress blisters by piercing and aspirating fluid 1, 4
• Apply nonadherent dressings (Mepitel™ or Telfa™) to denuded dermis with secondary foam dressings to collect exudate 1, 2
• Consider silver-containing products for sloughy areas only, guided by local microbiology 1

Infection Prevention and Management

• Do NOT use prophylactic systemic antibiotics - this increases skin colonization with resistant organisms, particularly Candida albicans 1, 2, 4
• Take swabs for bacterial and candidal culture from three lesional areas on alternate days 1
• Administer systemic antibiotics only when clinical signs of infection appear (confusion, hypotension, reduced urine output, reduced oxygen saturation, rising C-reactive protein) 1, 2, 4
• Monitor for monoculture of organisms on multiple site swabs indicating invasive infection 2

Nutrition Support

• Provide continuous enteral nutrition throughout the acute phase 1, 4
• Deliver 20-25 kcal/kg daily during the catabolic phase 1, 2
• Increase to 25-30 kcal/kg daily during the anabolic recovery phase 1
• Use nasogastric feeding when oral intake is precluded by buccal mucositis 4

Pain Management

• Use validated pain assessment tools at least once daily 1, 2
• Provide adequate background analgesia to ensure comfort at rest 1, 4
• Add supplementary opiates as required, delivered enterally or via patient-controlled analgesia 1, 2
• Consider sedation or general anesthesia for dressing changes and patient repositioning 2

Mucosal Management

Ophthalmologic Care (Critical)

• Arrange ophthalmology consultation within 24 hours of diagnosis - failure to do so leads to permanent visual impairment 2, 3, 4
• Perform daily examinations throughout the acute illness by ophthalmologist or trained nurse 4
• Apply preservative-free lubricant eye drops every 2 hours 4
• Perform daily ocular hygiene to remove inflammatory debris and break down conjunctival adhesions 4
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 4
• Consider topical corticosteroid drops under ophthalmologist supervision 4
• Consider amniotic membrane transplantation in acute phase for significantly better visual outcomes 4

Oral Care

• Apply white soft paraffin ointment to lips every 2 hours 4
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 4
• Apply antiseptic oral rinse twice daily 4
• Use topical anesthetics (viscous lidocaine 2% or cocaine mouthwashes 2-5%) for severe oral discomfort 4
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week 4

Urogenital Care

• Catheterize when urogenital involvement causes dysuria or retention 4
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 4
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae 4

Systemic Immunomodulatory Therapy

Evidence-Based Options

Cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month, has shown benefit with reduced mortality compared to predicted rates and is recommended as first-line immunomodulatory therapy. 3, 4, 5

Systemic corticosteroids (intravenous methylprednisolone 0.5-1 mg/kg) may be beneficial if started within 72 hours of onset, then converted to oral with tapering over at least 4 weeks. 3, 4 However, evidence is mixed - one comparison showed etanercept may reduce mortality compared to corticosteroids (though confidence intervals included possible harm). 6

Therapies with Equivocal or Negative Evidence

• IVIG has equivocal evidence - pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 4, 6
• Thalidomide should be avoided - associated with excess deaths in randomized trial 4
• Etanercept showed possible mortality reduction versus corticosteroids but evidence is low certainty 6

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients 1, 3
• Provide proton pump inhibitor if enteral nutrition cannot be established 1, 3
• Consider recombinant human G-CSF for neutropenic patients 1

Multidisciplinary Team Coordination

Care must be coordinated by a specialist in skin failure (dermatology or plastic surgery) with a team including:

• Intensive care physicians 1, 2
• Ophthalmologists 1, 2
• Specialist skincare nurses 1, 2
• Additional specialists as needed: respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, pharmacy 1, 2

Airway and Respiratory Management

• Respiratory symptoms and hypoxemia on admission require urgent intensivist discussion and rapid ICU transfer 4
• Perform fiberoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, and mechanically remove sloughed bronchial epithelium 4

Discharge Planning

• Provide written information about culprit drug(s) and potentially cross-reactive medications to avoid permanently 2, 3, 4
• Encourage wearing MedicAlert bracelet bearing the culprit drug name 2, 4
• Document drug allergy in medical records and inform all healthcare providers 2, 4
• Report adverse drug reaction to pharmacovigilance authorities 2, 4
• Arrange dermatology follow-up within weeks of discharge 2, 3
• Schedule ophthalmology follow-up to monitor for chronic ocular complications 2, 3
• Inform patients about potential fatigue and lethargy for several weeks requiring convalescence 4

Critical Pitfalls to Avoid

• Delayed transfer to specialized unit - significantly increases mortality 2, 4
• Continued use of culprit medication - worsens condition and increases mortality 4
• Indiscriminate prophylactic antibiotics - increases resistant organism colonization 1, 2, 4
• Overaggressive fluid resuscitation - causes pulmonary and tissue edema 2, 4
• Failure to involve ophthalmology within 24 hours - leads to permanent visual impairment 2, 3, 4
• Rough handling of skin - causes further epidermal detachment 1, 2