What is the appropriate management of Stevens‑Johnson syndrome?

Management of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit medications and transfer patients with >10% body surface area involvement to a specialized burn unit or ICU within hours—these two interventions are the most critical determinants of survival. 1, 2

Immediate Life-Saving Actions

• Stop all potential causative drugs on first suspicion of SJS, as this single intervention most directly impacts mortality 1, 2
• Calculate SCORTEN within 24 hours to predict mortality risk (scores 0-7 correlate with 1-99% mortality) and guide intensity of care 1, 2
• Transfer urgently to a burn center or ICU for patients with >10% BSA epidermal detachment—delayed transfer significantly increases mortality 1, 2, 3
• Arrange mandatory ophthalmology consultation within 24 hours of diagnosis; failure to do so leads to permanent visual impairment in 50-63% of patients 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
• Coordinate care through a multidisciplinary team including dermatology, intensive care, burn surgery, ophthalmology, and specialist skincare nurses 1, 2, 4

Wound and Skin Management

• Handle skin with extreme gentleness—any shearing force causes further epidermal detachment 1, 2, 3
• Leave detached epidermis in situ to act as a biological dressing; do not debride intact blisters 1, 2
• Apply greasy emollient (50% white soft paraffin + 50% liquid paraffin) over the entire epidermis including denuded areas every 2-4 hours 1, 2
• Cleanse wounds gently using warmed sterile water, saline, or dilute chlorhexidine (1:5000) 1, 2
• Apply nonadherent dressings to denuded dermis with secondary foam or burn dressings to collect exudate 1, 3
• Consider high-strength topical corticosteroids on affected skin areas as part of wound care 1

Infection Prevention and Management

Do not use prophylactic systemic antibiotics—this is a critical pitfall that increases skin colonization with resistant organisms, particularly Candida 1, 2, 5

• Take swabs for bacterial and candidal culture from three lesional areas on alternate days 1, 2
• Monitor for clinical infection signs: confusion, hypotension, oliguria, desaturation, increased skin pain, rising CRP, neutrophilia 1
• Administer targeted antimicrobial therapy only when clinical signs of infection appear 1, 2
• Recognize that sepsis is the leading cause of death, especially when antibiotics are the causative agent 6

Mucosal Management

Ocular Care

• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 1, 2, 3
• Perform daily ocular hygiene by an ophthalmologist or 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, 3
• Apply topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 1, 3
• Continue daily ophthalmological reviews throughout the acute illness 1, 2, 3

Oral Care

• Apply white soft paraffin ointment to lips every 2 hours to reduce risk of fibrotic scars 1, 2, 3
• Use benzydamine hydrochloride anti-inflammatory oral rinse every 3 hours, particularly before eating 1, 3
• Perform antiseptic oral rinse (chlorhexidine) twice daily to reduce bacterial colonization 1, 3
• Provide viscous lidocaine 2% or cocaine mouthwash (2-5%) for severe oral discomfort 1
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week 1

Urogenital Care

• Apply white soft paraffin ointment to urogenital skin and mucosa every 4 hours 1, 3
• Use urinary catheterization when urogenital involvement causes dysuria, retention, or when accurate output monitoring is required 1
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1
• Apply potent topical corticosteroid ointment once daily to involved, non-eroded surfaces 3

Fluid, Electrolyte, and Nutritional Support

• Establish adequate IV fluid replacement guided by urine output and hemodynamic parameters—avoid overaggressive resuscitation that causes pulmonary, cutaneous, and intestinal edema 1, 2, 3
• Monitor vital signs, urine output, and electrolytes regularly 1
• Provide continuous enteral nutrition: 20-25 kcal/kg/day during catabolic phase, increasing to 25-30 kcal/kg/day during recovery 1, 2
• Consider nasogastric feeding when oral intake is precluded by buccal mucositis 1

Pain Management

• Provide adequate background simple analgesia to ensure comfort at rest 1
• Add opioid analgesia for breakthrough pain episodes 1
• Use validated pain assessment tools at least once daily 1

Systemic Immunomodulatory Therapy

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, 7, 5

• If using corticosteroids, start early (within 72 hours) with IV methylprednisolone 0.5-1 mg/kg or oral prednisolone 1-2 mg/kg/day, followed by conversion to oral with taper lasting at least 4 weeks 1, 2, 3
• For immune-checkpoint-inhibitor-induced SJS: use methylprednisolone 0.5-1 mg/kg for Grade 3 disease or 1-2 mg/kg for Grade 4 disease, and permanently discontinue the offending agent 1
• IVIG may be considered in severe disease or inadequate response to corticosteroids, though pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 1, 7, 5
• Avoid thalidomide—it was associated with excess deaths in one randomized trial 1

Airway and Respiratory Management

• Respiratory symptoms and hypoxemia on admission require urgent intensivist discussion and rapid ICU/burn center transfer 1, 2, 3
• Perform fiberoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, and mechanically remove sloughed bronchial epithelium 1
• Intubate and ventilate only in extreme circumstances—mechanical ventilation is a high-risk mortality factor 1, 6

Pediatric Considerations

• Infectious triggers account for up to 50% of pediatric SJS/TEN—test for Mycoplasma pneumoniae and herpes simplex virus 1, 2
• Consult infectious disease team in all pediatric cases and consider targeted antibiotics (e.g., azithromycin for mycoplasma) 1
• Pediatric mortality is lower (0-8.5%) but recurrence occurs in up to 18% of cases 1
• Ophthalmology assessment within 24 hours is mandatory to avoid permanent visual sequelae, the most common long-term complication in children 1

Discharge Planning and Long-Term Follow-Up

• Provide written information about the culprit drug(s) and any cross-reactive medications 1, 2, 3
• Advise patients to wear a MedicAlert bracelet naming the culprit drug 1, 2, 3
• Document drug allergy in medical records and communicate to all healthcare providers 1, 2, 3
• Report the adverse drug reaction to national pharmacovigilance authorities 1, 2, 3
• Counsel that fatigue and lethargy may persist for several weeks after discharge 1, 2
• Schedule ophthalmology follow-up to monitor for chronic ocular complications 2
• Consider referral to support groups such as SJS Awareness U.K. 1

Critical Pitfalls to Avoid

• Examining the patient without awareness of skin fragility causes additional epidermal detachment—warn all clinicians of this risk 1
• Delayed recognition and transfer significantly increases mortality 1, 2, 3
• Prophylactic antibiotics increase resistant organism colonization 1, 2
• Overaggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema 1, 2, 3
• Failure to involve ophthalmology early results in permanent visual sequelae 1, 2, 3
• Continued use of the culprit medication worsens the condition and increases mortality 1
• Age over 65 years and mechanical ventilation requirement are high-risk mortality factors 6