What is the appropriate management and treatment for Stevens‑Johnson syndrome?

Management of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit drugs and transfer the patient to a specialized burn unit or ICU within hours of diagnosis, particularly when body surface area involvement exceeds 10%, as delayed transfer significantly increases mortality risk. 1, 2

Immediate Life-Saving Actions

• Stop all potential causative medications immediately – this is the single most critical intervention that directly impacts survival 1, 2
• Calculate SCORTEN within the first 24 hours to predict mortality risk (scores range 0-7, with mortality from 1% to 99%) 1, 2, 3
• Transfer urgently to a burn center or ICU with multidisciplinary expertise including dermatology, intensive care, ophthalmology, and specialized wound care nursing 1, 2
• Barrier-nurse the patient in a temperature-controlled room (25-28°C) on a pressure-relieving mattress to prevent hypothermia and reduce infection risk 1, 2

Supportive Care Framework

Fluid Management

• Establish intravenous fluid replacement guided by urine output and hemodynamic parameters, avoiding overaggressive resuscitation that causes pulmonary, cutaneous, and intestinal edema 1, 2
• Insert urinary catheter for accurate monitoring when urogenital involvement causes dysuria or retention 1, 2

Wound Care Protocol

• Handle skin with extreme gentleness to minimize shearing forces that cause further epidermal detachment 1, 2, 4
• Leave detached epidermis in situ to act as a biological dressing rather than removing it 1, 2, 4
• Irrigate wounds gently using warmed sterile water, saline, or 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 1, 2, 4
• Apply nonadherent dressings to denuded dermis with secondary foam or burn dressings to collect exudate 1, 2, 4
• Consider silver-containing dressings only for sloughy areas 1

Nutrition Support

• 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 2
• Use nasogastric feeding when oral intake is precluded by severe buccal mucositis 2

Pain Management

• Provide adequate background analgesia with simple analgesics to ensure comfort at rest 1, 2
• Add opioid analgesia for breakthrough pain, particularly during wound care procedures 1, 2
• Use validated pain assessment tools at least once daily 2

Critical Mucosal Management

Ophthalmological Care (Most Important for Long-Term Quality of Life)

• Arrange ophthalmology consultation within 24 hours of diagnosis with daily examinations throughout the acute phase – failure to do this leads to permanent visual impairment 1, 2, 5, 4
• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 1, 2, 5, 4
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 1, 2, 5, 4
• Apply topical antibiotics when corneal fluorescein staining or ulceration is present 1, 2, 4
• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 1, 2, 5
• Consider amniotic membrane transplantation in the acute phase for significantly better visual outcomes 2

Oral Care

• Perform daily oral examination during the acute illness 1, 2, 4
• Apply white soft paraffin ointment to the lips every 2 hours 1, 2, 4
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 1, 2, 4
• Use antiseptic oral rinse containing chlorhexidine twice daily to reduce bacterial colonization 1, 2, 4
• Apply topical anesthetics such as viscous lidocaine 2% or cocaine mouthwashes 2-5% for severe oral discomfort 2
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 2

Urogenital Care

• Perform daily urogenital examination during the acute illness 1, 2, 4
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1, 2, 4
• Apply potent topical corticosteroid ointment once daily to involved, noneroded surfaces 2, 4
• Use silicone dressings (e.g., Mepitel) to eroded areas 2, 4
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 2

Systemic Immunomodulatory Therapy

Administer IV methylprednisolone 0.5-1 mg/kg (or equivalent) if started within 72 hours of onset, converting to oral corticosteroids on response with tapering over at least 4 weeks. 1, 2, 5, 6

• The FDA label specifically indicates methylprednisolone for "severe erythema multiforme (Stevens-Johnson syndrome)" 6
• For immune checkpoint inhibitor-induced SJS/TEN (grade 3-4), use IV methylprednisolone 1-2 mg/kg, tapering when toxicity resolves 1
• Alternative option: Cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month – multiple studies show reduced mortality compared to predicted rates 1, 2, 5, 4
• Consider IVIG or cyclosporine in severe or steroid-unresponsive cases, though evidence for IVIG is equivocal (pooled analysis showed no mortality benefit, OR 1.00,95% CI 0.58-1.75) 1, 2
• Avoid thalidomide – one randomized trial showed excess deaths 2

Evidence Nuances on Systemic Therapy

The guidelines show divergent evidence on systemic immunomodulation. The British guidelines 1 suggest cyclosporine has the strongest evidence base with consistent mortality reduction across multiple studies. Early corticosteroids (within 72 hours) show benefit in several studies 1, 2, 5, though this remains somewhat controversial. IVIG, despite widespread use, lacks robust mortality benefit in pooled analyses 2. In the context of immune checkpoint inhibitor-induced SJS/TEN, corticosteroids are specifically recommended as the underlying mechanism is T-cell immune-directed toxicity 1.

Infection Prevention and Management

• Do NOT use prophylactic systemic antibiotics – this increases skin colonization with resistant organisms, particularly Candida albicans 1, 2, 5, 4
• Take swabs for bacterial and candidal culture from three lesional areas on alternate days 1, 2
• Monitor for rising C-reactive protein and neutrophilia as indicators of sepsis 2
• Institute targeted antimicrobial therapy ONLY when clinical signs of infection appear (fever with hemodynamic instability, monoculture of organisms from multiple sites) 1, 2, 5, 4

Airway Management

• Respiratory symptoms and hypoxemia on admission require urgent intensivist discussion and rapid ICU transfer 1, 2, 4
• 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

Special Considerations for Pediatric Patients

• Infection causes up to 50% of pediatric SJS/TEN cases (versus drugs in adults) – test for Mycoplasma pneumoniae, Chlamydia, HSV, and varicella zoster virus 1, 2, 5
• Consult infectious disease team in all pediatric cases and consider targeted antibiotics (e.g., azithromycin for mycoplasma) 2
• Manage in age-appropriate specialist units with pediatric intensivists and skin loss specialists 2, 5
• Mortality is lower in children (0% for SJS, 8-8.5% for TEN) compared to adults (3-31% for SJS, 15-42% for TEN) 1
• Recurrence is more common in children (up to 18%) because the precipitant is usually infection rather than drugs 1

Discharge Planning and Long-Term Follow-Up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 1, 2, 5, 4
• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug 1, 2, 5, 4
• Document drug allergy in the patient's medical records and inform all healthcare providers, especially the GP 1, 2, 5, 4
• Report the adverse drug reaction to pharmacovigilance authorities (MHRA in the U.K.) 1, 2, 5, 4
• Refer to specialist drug allergy service for expert review 1
• Arrange dermatology outpatient appointment within a few weeks of discharge 4
• Arrange ophthalmology follow-up if ocular involvement occurred 4
• Inform patients about expected fatigue and lethargy for several weeks following discharge, requiring convalescence 1, 2, 5
• Consider referral to support groups such as SJS Awareness U.K. 1, 2, 5
• Refer to local social services for needs assessment when appropriate 1

Critical Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality – transfer should occur within hours, not days 1, 2, 5
• Failure to involve ophthalmology within 24 hours leads to permanent visual impairment – ocular damage is the most disabling long-term complication 1, 2, 5
• Indiscriminate use of prophylactic antibiotics increases resistant organism colonization 1, 2, 5, 4
• Overaggressive fluid resuscitation causes pulmonary, cutaneous, and intestinal edema 1, 2, 5
• Continued use of the culprit medication will worsen the condition and increase mortality 1, 2, 5, 4
• Removing detached epidermis rather than leaving it in situ as a biological dressing 1, 2, 4
• Failure to calculate SCORTEN for mortality risk stratification 1, 2, 3