How should Stevens‑Johnson syndrome be managed, including immediate drug cessation, supportive care, and indications for systemic immunomodulatory therapy?

How to Treat Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit medications, transfer patients with >10% body surface area involvement to a specialized burn unit or ICU within hours, and initiate comprehensive supportive care as the cornerstone of management—this approach directly impacts survival. 1, 2

Immediate Life-Saving Actions

• Stop all potential causative drugs the moment SJS is suspected—this single intervention most directly impacts survival 1, 2
• Calculate SCORTEN within 24 hours to predict mortality (scores 0-7 correspond to 1-99% mortality) and guide intensity of care 1, 2
• Transfer without delay to a specialized burn center or ICU staffed by multidisciplinary teams (dermatology, intensive care, ophthalmology, specialized nursing) when body surface area detachment exceeds 10%—delayed transfer markedly increases mortality 1, 2
• Arrange mandatory ophthalmology consultation within 24 hours of diagnosis; failure to do so is associated with permanent visual impairment 1, 2

Specialized Care Environment

• 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
• Ensure daily bedside care is performed by specialist nurses experienced with skin-fragility disorders 1

Skin and Wound Management

Handling Principles

• Handle skin with extreme gentleness using anti-shear techniques to minimize further epidermal detachment—examining patients without awareness of skin fragility can cause additional damage 1, 2
• Leave detached epidermis in situ to act as a biological dressing; do not debride intact blisters 1, 2
• Decompress blisters by piercing and expressing or aspirating fluid 1

Wound Care Protocol

• Cleanse wounds gently by irrigating with warmed sterile water, saline, or dilute chlorhexidine (1:5000) 1, 2
• Apply greasy emollient (50% white soft paraffin + 50% liquid paraffin) over the entire epidermis including denuded areas every 2-4 hours to support barrier function and reduce trans-epidermal water loss 1, 2
• Use non-adherent dressings (e.g., Mepitel or Telfa) over denuded dermis, covered with secondary foam or burn dressings to collect exudate 1
• Apply silver-containing dressings only to sloughy areas, not routinely 1
• Consider high-strength topical corticosteroids (clobetasol, betamethasone) on affected skin areas 3, 1

Infection Prevention and Management

Critical Pitfall: Do not use prophylactic systemic antibiotics—indiscriminate administration increases skin colonization with resistant organisms, particularly Candida albicans, without improving outcomes 1, 2, 4

• Obtain bacterial and candidal swabs from three lesional sites on alternate days throughout the acute phase 1, 2
• Monitor closely for infection signs: confusion, hypotension, oliguria, desaturation, increased skin pain, rising C-reactive protein, and neutrophilia 1
• Initiate targeted antimicrobial therapy only when clinical infection signs are present—appearance of monoculture from multiple sites signals predominant strain and raises risk of invasive infection 1, 2
• Consider herpes simplex virus reactivation in slowly healing eroded or vesicular areas, especially genital and oral sites 1

Fluid, Electrolyte, and Nutritional Support

• Establish adequate intravenous fluid replacement guided by urine output and hemodynamic parameters, avoiding over-aggressive resuscitation that causes pulmonary, cutaneous, and intestinal edema 1, 2
• Monitor fluid balance regularly using vital signs, urine output, and electrolyte measurements 1, 2
• Provide continuous enteral nutrition: 20-25 kcal/kg/day during the catabolic phase, increasing to 25-30 kcal/kg/day during recovery 1, 2
• Use nasogastric feeding when oral intake is impossible due to buccal mucositis 1

Mucosal Management

Ophthalmologic Care (Critical for Preventing Permanent Sequelae)

• Perform ophthalmology examination within 24 hours and repeat daily throughout the acute phase—50-63% develop late complications including severe dry eyes and trichiasis 1, 2
• Apply preservative-free lubricating eye drops every 2 hours throughout the acute illness 1, 2
• Conduct daily ocular hygiene by an ophthalmologist or trained nurse to remove inflammatory debris and break conjunctival adhesions 1
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 1
• Apply topical corticosteroid eye drops under ophthalmology supervision to reduce ocular surface damage 1

Oral Care

• Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute phase to reduce risk of fibrotic scars 1, 2
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, especially before meals 1, 2
• Perform antiseptic oral rinse twice daily to lower bacterial colonization 1
• Provide topical anesthetic (viscous lidocaine 2% or cocaine mouthwash 2-5%) for severe oral discomfort 1
• Apply topical corticosteroids to oral mucosa as needed 1

Urogenital Care

• Insert urinary catheter when urogenital involvement causes dysuria, retention, or to monitor output 1
• Apply white soft paraffin ointment to urogenital skin and mucosa every 4 hours 1
• Use vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1

Pain Management

• Provide baseline non-opioid analgesia to ensure comfort at rest 1
• Add opioid analgesia for breakthrough pain 3, 1

Systemic Immunomodulatory Therapy

First-Line Recommendation

Cyclosporine 3 mg/kg daily for 10 days (tapered over 1 month) has shown the most consistent benefit with reduced mortality compared to predicted rates in multiple studies 1, 2

Alternative: Early Corticosteroids

• If using corticosteroids, start within 72 hours of onset with IV methylprednisolone 0.5-1 mg/kg (or oral prednisolone 1-2 mg/kg/day), then convert to oral corticosteroids with a taper lasting at least 4 weeks 3, 1, 5
• Early methylprednisolone pulse therapy may be beneficial if initiated within 72 hours 1

Evidence Considerations

• Etanercept may reduce mortality compared to corticosteroids (low-certainty evidence from one study with 91 participants) 6
• IVIG evidence is equivocal—pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 1, 6
• Thalidomide should be avoided—associated with excess deaths in one randomized trial 1

For Immune Checkpoint Inhibitor-Induced SJS/TEN

• Permanently discontinue the offending immune checkpoint inhibitor 3
• Use IV methylprednisolone 1-2 mg/kg for grade 4 disease or 0.5-1 mg/kg for grade 3 disease 3
• Corticosteroids are not contraindicated in this setting because pathology is driven by T-cell-mediated immune toxicity 3

Airway and Respiratory Management

• Respiratory symptoms and hypoxemia on admission require urgent intensivist discussion and rapid ICU/burn center transfer 1, 2
• Perform fibreoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, investigate pneumonitis, and mechanically remove sloughed bronchial epithelium 1
• Intubate and ventilate only in extreme circumstances due to complications including nosocomial pneumonia and fluid overload 2

Pediatric Considerations

• Infectious triggers account for up to 50% of pediatric SJS/TEN—test for Mycoplasma pneumoniae and herpes simplex virus and involve infectious disease specialists 1
• Mortality in children is lower than adults (0-8.5% depending on severity), but recurrence occurs in up to 18% of cases 1
• Ensure ophthalmology assessment within 24 hours to avoid permanent visual sequelae, which are the most common long-term complication in children 1

Discharge Planning and Follow-Up

• Provide written information about the culprit drug(s) and any cross-reactive medications to avoid 1, 2
• Advise patients to wear a MedicAlert bracelet naming the culprit drug 1, 2
• Document the drug allergy in the medical record and inform all healthcare providers 1, 2
• Report the adverse drug reaction to national pharmacovigilance authorities 1, 2
• Counsel patients that fatigue and lethargy may persist for several weeks after discharge and that convalescence is required 1
• Offer referral to support groups such as SJS Awareness U.K. 1
• Schedule ophthalmology follow-up to monitor for chronic ocular complications 2

Critical Pitfalls to Avoid

• Delayed transfer to specialized care significantly increases mortality 1, 2
• Indiscriminate prophylactic antibiotics increase resistant organism colonization without improving outcomes 1, 2, 4
• Continued use of the culprit medication worsens the condition and raises mortality 1
• Over-aggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema 1, 2
• Failure to involve ophthalmology early can lead to permanent visual sequelae 1, 2
• Examining patients without awareness of skin fragility can cause additional epidermal detachment 1