What is the appropriate management for a patient with Stevens‑Johnson syndrome or toxic epidermal necrolysis?

Management of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Immediately discontinue all suspected culprit drugs and transfer patients with >10% body surface area epidermal detachment to a specialized burn center or ICU within hours of diagnosis, as delayed transfer significantly increases mortality. 1, 2

Initial Assessment and Triage

• Calculate SCORTEN within the first 24 hours to predict mortality risk (scores 0-7 correspond to mortality rates of 1-99%) and guide intensity of care 1, 2
• Document the exact date of rash onset, progression pattern, and all medications taken in the preceding 2 months including over-the-counter and herbal products 2
• Obtain skin biopsy showing confluent epidermal necrosis with subepidermal vesicle formation to confirm diagnosis 2
• Arrange mandatory ophthalmology consultation within 24 hours of diagnosis, as failure to do so is associated with permanent visual impairment 2

Specialized Care Environment

• Admit patients to a burn center or ICU with multidisciplinary expertise including dermatology, intensive care, ophthalmology, and specialist skincare nursing 1, 3, 4
• Provide barrier nursing in a temperature-controlled room (25-28°C) on a pressure-relieving mattress to prevent hypothermia and reduce infection risk 1, 2
• Early transfer to specialized centers reduces mortality; delays adversely affect outcomes 1, 2, 3

Skin and Wound Management

Handling and Basic Care

• Handle skin with extreme gentleness using antishear techniques to minimize forces that cause further epidermal detachment 1, 2
• Daily bedside care should be performed by specialist nurses familiar with skin fragility disorders 1
• Warn all examining clinicians unfamiliar with epidermal detachment problems before they examine the patient 2

Wound Cleansing and Dressing

• Cleanse wounds gently by irrigating with warmed sterile water, saline, or dilute chlorhexidine (1:5000) 1, 2
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis including denuded areas every 2-4 hours to support barrier function, reduce transcutaneous water loss, and encourage re-epithelialization 1, 2
• Leave detached epidermis in situ to act as a biological dressing 1, 2
• Decompress blisters by piercing and expressing or aspirating fluid 1
• Apply nonadherent dressings (such as Mepitel or Telfa) to denuded dermis with secondary foam or burn dressings to collect exudate 1, 2
• Consider silver-containing products or dressings for sloughy areas only 1
• Modern nanocrystalline dressings may be kept in situ for longer periods, reducing pain and morbidity from frequent dressing changes 3

Surgical Approach

• Reserve surgical debridement of detached epidermis followed by biosynthetic xenograft or allograft for cases with clinical deterioration, extension of epidermal detachment, local sepsis/subepidermal pus, delayed healing, or wound conversion 1, 2

Infection Prevention and Management

Do not use prophylactic systemic antibiotics, as indiscriminate administration increases skin colonization with resistant organisms, particularly Candida albicans. 1, 2

• Take swabs for bacterial and candidal culture from three lesional areas on alternate days 1, 2
• Monitor carefully for clinical signs of infection: confusion, hypotension, reduced urine output, reduced oxygen saturation, increased skin pain, rising C-reactive protein, and neutrophilia 1, 2
• A monoculture of organisms detected on culture from multiple sites that previously showed mixed growth indicates one strain is becoming predominant and increases likelihood of invasive infection 1
• Administer systemic antibiotics only when clinical signs of infection are present 1, 2
• Consider activation of HSV in eroded or vesicular areas that are slow to heal, particularly in genital and oral sites 1

Fluid, Electrolyte, and Nutritional Support

• Establish adequate intravenous fluid replacement guided by urine output and hemodynamic parameters to prevent end-organ hypoperfusion 2
• Avoid overaggressive fluid resuscitation that leads to pulmonary, cutaneous, and intestinal edema 1, 2
• Monitor fluid balance with regular assessment of vital signs, urine output, and electrolytes 1, 2
• Provide continuous enteral nutrition throughout the acute phase: 20-25 kcal/kg daily during the catabolic phase, increasing to 25-30 kcal/kg during recovery 1, 2
• Consider nasogastric feeding when oral intake is precluded by buccal mucositis 1

Mucosal Management

Ophthalmologic Care

• Ophthalmology examination within 24 hours of diagnosis with daily reviews throughout the acute illness 1, 2
• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 1, 2
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 1, 2
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 1, 2
• Apply topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 1, 2
• Consider amniotic membrane transplantation in the acute phase, which demonstrates significantly better visual outcomes compared to medical management alone 2

Oral Care

• Examine the mouth as part of initial assessment with daily oral review during acute illness 2
• Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness 1, 2
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 1, 2
• Perform antiseptic oral rinse twice daily to reduce bacterial colonization 1, 2
• Provide topical anesthetics such as viscous lidocaine 2% or cocaine mouthwashes 2-5% for severe oral discomfort 1, 2
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 2
• Apply topical corticosteroids to oral mucosa 1

Urogenital Care

• Perform urinary catheterization when urogenital involvement causes dysuria or retention, or to monitor output 1, 2
• Regular examination of urogenital tract during acute illness 1
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1, 2
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1, 2

Pain Management

• Use validated pain assessment tools at least once daily 2
• Provide adequate background simple (non-opioid) analgesia to ensure comfort at rest 1, 2
• Add opioid analgesia for breakthrough pain 1, 2
• High-strength topical corticosteroids may be applied to affected skin areas as part of pain control 2

Systemic Immunomodulatory Therapy

Corticosteroids

• Intravenous methylprednisolone 0.5-1 mg/kg (or equivalent corticosteroid) initiated within 72 hours of disease onset, followed by conversion to oral corticosteroids with a taper lasting at least 4 weeks 1, 2
• Early methylprednisolone pulse therapy may be beneficial if started within 72 hours 1

Cyclosporine

• Cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month, has shown benefit in multiple studies with reduced mortality compared to predicted rates 1

Other Agents

• IVIG may be considered in patients with severe disease or those who do not respond adequately to corticosteroids, though pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 2
• Thalidomide (an anti-TNF agent) was associated with excess deaths in one randomized trial and should be avoided 2

Immune Checkpoint Inhibitor-Induced SJS/TEN

• Permanent discontinuation of the offending immune checkpoint inhibitor is required 2
• For Grade 3 disease: use methylprednisolone 0.5-1 mg/kg 2
• For Grade 4 disease: use methylprednisolone 1-2 mg/kg 2
• Corticosteroids are not contraindicated in this setting because the pathology is driven by T-cell-mediated immune toxicity 2

Airway and Respiratory Management

• Respiratory symptoms and hypoxemia on admission should prompt urgent discussion with an intensivist and rapid transfer to ICU or burn center 2
• Perform fiberoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, investigate pneumonitis, and mechanically remove sloughed bronchial epithelium 2

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients 2
• Provide proton pump inhibitor if enteral nutrition cannot be established 2
• Consider recombinant human G-CSF for neutropenic patients 2

Pediatric Considerations

• Infectious triggers (particularly Mycoplasma pneumoniae and HSV) account for up to 50% of pediatric SJS/TEN cases 2
• Test for infective triggers and consult infectious disease team in all pediatric cases 2
• Consider targeted antibiotics as appropriate (e.g., azithromycin for mycoplasma) 2
• Manage pediatric patients in age-appropriate specialist units with pediatric intensivists and skin loss specialists 2
• Mortality in children is lower than in adults (0-8.5% depending on severity), but recurrence occurs in up to 18% of cases 2
• Ophthalmology assessment within 24 hours is critical, as permanent visual sequelae are the most common long-term complication in children 2

Discharge Planning and Follow-Up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 1, 2
• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug 1, 2
• Document the drug allergy in the patient's medical records and inform all healthcare providers involved in their care 1, 2
• Report the adverse drug reaction to national pharmacovigilance authorities 1, 2
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 1, 2
• Consider referral to support groups such as SJS Awareness U.K. 1, 2

Critical Pitfalls to Avoid

• Delayed transfer to specialized care significantly increases mortality 1, 2, 3
• Examining the patient without awareness of skin fragility can cause additional epidermal detachment; all clinicians must be warned of this risk 2
• Indiscriminate prophylactic antibiotics increase resistant organism colonization and do not improve outcomes 1, 2
• Failure to involve ophthalmology within 24 hours leads to permanent visual impairment 2
• Continued use of the culprit medication will worsen the condition and increase mortality 1, 2
• Overaggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema 1, 2