What is the treatment for Steven's Johnson syndrome?

Treatment of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit drugs and transfer the patient to a specialized burn unit or ICU with multidisciplinary SJS/TEN experience, particularly when body surface area involvement exceeds 10%, while initiating comprehensive supportive care as the cornerstone of management. 1, 2, 3

Initial Assessment and Triage

Calculate SCORTEN on admission to predict mortality risk and guide intensity of care—this severity scoring system should be performed within 24 hours of presentation. 1, 2, 3

Transfer criteria to specialized centers include: 1

• Confirmed TEN (>30% skin detachment)
• SJS/TEN overlap (10-30% involvement) with poor prognostic factors
• Severe eye disease requiring specialized interventions like amniotic membrane transplant
• Any patient with >10% body surface area epidermal detachment 2, 3

Barrier nursing in temperature-controlled environment (25-28°C) on pressure-relieving mattress to minimize further skin trauma. 1, 2

Supportive Care: The Foundation of Treatment

Fluid Management

Establish adequate intravenous fluid replacement guided by urine output and end-organ perfusion markers (serum lactate, base deficit, urea, electrolytes), while carefully avoiding fluid overload that causes pulmonary, cutaneous, and intestinal edema. 1, 2

• Monitor fluid balance with urinary catheterization when clinically indicated 1
• Use continuous invasive hemodynamic monitoring through central or arterial lines in severely affected cases 1
• Adjust fluid replacement daily with careful sodium monitoring 1
• Change peripheral IV cannulas every 2-3 days through non-lesional skin; central lines every 5-7 days 1

Nutritional Support

Deliver continuous enteral nutrition throughout the acute phase: 20-25 kcal/kg daily during the catabolic phase and 25-30 kcal/kg during recovery, using nasogastric feeding when oral intake is precluded by buccal mucositis. 2

Skin and Wound Care

Handle skin with extreme care to minimize shearing forces that extend epidermal detachment—this is critical to prevent worsening of the condition. 1, 2, 3

Specific wound management techniques: 1, 2

• Leave detached epidermis in situ to act as biological dressing 2, 3
• Decompress blisters by piercing and expressing fluid 2
• Apply greasy emollients (white soft paraffin) over entire epidermis including denuded areas 2, 3
• Use nonadherent dressings (e.g., Mepitel) to denuded dermis with secondary foam or burn dressings to collect exudate 2, 3
• Consider silver-containing dressings for sloughy areas only 2
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 2

Minimize iatrogenic trauma: 1

• Avoid sphygmomanometer cuffs, adhesive ECG leads, adhesive dressings, and identification wrist tags
• Place thin soft clothing under blood pressure cuffs
• Cover fingertips with clingfilm before attaching pulse oximetry monitors
• Use soft silicone tapes for essential clinical items (cannulas, nasogastric tubes)
• Use silicone medical adhesive remover for adherent clothes or dressings

Pain Management

Administer adequate analgesia using intravenous opioid infusions in those not tolerating oral medication, with patient-controlled analgesia where appropriate. 1

• Assess pain at least once daily using validated pain tools in conscious patients 1
• Provide adequate background simple analgesia with additional opioid analgesia for breakthrough pain 2
• Consider sedation or general anesthesia for patient handling, repositioning, and dressing changes 1
• Consider keeping severely affected patients sedated and ventilated in ICU for duration of acute phase 1

Infection Prevention and Management

Do NOT use prophylactic antibiotics—this is a critical pitfall that increases skin colonization with resistant organisms, particularly Candida. 2, 3, 4

Monitor for clinical signs of infection rather than treating prophylactically: 2, 3

• Take swabs for bacterial and candidal culture from lesional skin, particularly sloughy or crusted areas, throughout the acute phase 1
• Take viral swabs from eroded areas if HSV infection is suspected 1
• Institute targeted antimicrobial therapy only when clinical signs of infection appear 2, 3
• Recognize that fever from SJS/TEN itself complicates detection of secondary sepsis 3

Mucosal Management

Ocular Care (Critical for Preventing Permanent Sequelae)

Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews during acute illness—this is non-negotiable as delayed ophthalmology involvement leads to permanent visual impairment. 1, 2, 3

Specific ocular interventions: 1, 2, 3

• Apply preservative-free lubricant eye drops every 2 hours throughout acute illness
• Perform daily ocular hygiene by ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions
• Use topical antibiotics when corneal fluorescein staining or ulceration is present
• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage
• Consider amniotic membrane transplantation in acute phase for significantly better visual outcomes 2

Oral Care

Apply white soft paraffin ointment to lips immediately, then every 2 hours throughout acute illness to reduce risk of fibrotic scars. 2, 3

Additional oral management: 2, 3

• Daily oral review during acute illness
• Anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating
• Antiseptic oral rinse containing chlorhexidine twice daily
• Clean mouth daily with warm saline mouthwashes or oral sponge
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 2
• Consider topical anesthetics (viscous lidocaine 2% or cocaine mouthwashes 2-5%) for severe oral discomfort 2

Urogenital Care

Daily urogenital review with application of white soft paraffin ointment to urogenital skin and mucosae every 4 hours. 2, 3, 5

Specific urogenital interventions: 2, 3, 5

• Catheterization when urogenital involvement causes dysuria or retention, or to monitor output and prevent urethral strictures
• Use potent topical corticosteroid ointment once daily to involved, non-eroded surfaces
• Apply silicone dressings (e.g., Mepitel) to eroded areas
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation
• Early evaluation by vulvar specialist for women 5

Systemic Immunomodulatory Therapy

The evidence for specific immunomodulatory agents remains debated, with no definitive high-quality randomized controlled trials establishing superiority of one agent over another. 6, 7, 4 However, based on available evidence:

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 and may be used alone or in combination with corticosteroids. 2, 3, 6

Systemic corticosteroids (prednisolone 1-2 mg/kg/day or equivalent, or IV methylprednisolone pulse therapy) may be beneficial if started early, preferably within 72 hours of onset, tapered rapidly within 7-10 days. 2, 3, 8, 6 The FDA label indicates corticosteroids for severe erythema multiforme (Stevens-Johnson syndrome). 8 If steroids were initiated at an outside facility, consider tapering and discontinuing based on individual assessment. 9

Intravenous immunoglobulin (IVIG) is frequently used, though evidence quality varies. 6, 7, 9

The British Journal of Dermatology guidelines recommend cyclosporine as the preferred systemic agent based on available evidence, with corticosteroids as an alternative if started within 72 hours. 2, 3

Airway and Respiratory Management

Respiratory symptoms and hypoxemia on admission should prompt early discussion with intensivist and rapid transfer to ICU or burn center. 3

• Perform fiberoptic bronchoscopy to assess airway involvement 3
• Mechanical ventilation only in extreme circumstances due to complications (nosocomial pneumonia, fluid overload) 1

Discharge Planning and Follow-up

Provide written information about culprit drug(s) to avoid and potentially cross-reactive medications. 2, 3

Essential discharge actions: 2, 3

• Encourage patient to wear MedicAlert bracelet bearing name of culprit drug
• Document drug allergy in patient's medical records and inform all healthcare providers
• Report adverse drug reaction to national pharmacovigilance authorities
• Organize dermatology outpatient appointment within few weeks of discharge
• Arrange ophthalmology follow-up if ocular involvement occurred
• Inform patients about potential fatigue and lethargy for several weeks following discharge
• Consider referral to support groups (e.g., SJS Awareness U.K.)

Special Considerations for Pediatric Patients

Infection causes up to 50% of pediatric SJS/TEN cases—test for infective triggers and consult infectious disease team in all pediatric cases. 3

• Manage in age-appropriate specialist units with pediatric intensivists and skin loss specialists 3
• High-risk children need quicker transfer to specialized care 3

Common Pitfalls to Avoid

Delayed recognition and transfer to specialized care significantly increases mortality—early transfer reduces mortality and improves outcomes. 3, 4

Indiscriminate prophylactic antibiotics increase resistant organism colonization, particularly Candida—this is one of the most common and harmful errors in SJS/TEN management. 2, 3

Overaggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema—careful monitoring is essential. 2, 3

Failure to involve ophthalmology within 24 hours leads to permanent visual sequelae—this consultation is mandatory, not optional. 2, 3

Continued use of culprit medication worsens condition and increases mortality—immediate discontinuation of all suspected drugs is the single most important intervention. 2, 3