What is the treatment for Steven 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 expertise, particularly when body surface area involvement exceeds 10%. 1, 2

Initial Assessment and Triage

• Calculate SCORTEN on admission to predict mortality risk and guide intensity of care 1, 2
• Obtain skin biopsy to confirm diagnosis, looking for confluent epidermal necrosis with subepidermal vesicle formation 1
• Document exact date of rash onset and all medications taken in the previous 2 months, including over-the-counter and herbal products 1
• Early transfer to specialized centers reduces mortality; delays in specialized care adversely affect outcomes 1

Supportive Care Framework

Fluid and Nutritional Management

• Provide careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload that can lead to pulmonary, cutaneous, and intestinal edema 1, 2
• Monitor fluid balance with regular assessment of vital signs, urine output, and electrolytes 1
• Deliver continuous enteral nutrition throughout the acute phase: 20-25 kcal/kg daily during the catabolic phase and 25-30 kcal/kg during recovery 1
• Consider nasogastric feeding when oral intake is precluded by buccal mucositis 1

Wound Management

• Barrier nurse patients in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 1, 2
• Minimize shearing forces when handling skin to prevent further epidermal detachment 1, 2
• Leave detached epidermis in situ to act as a biological dressing 1, 2
• Decompress blisters by piercing and expression or aspiration of fluid 1
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 1
• Apply greasy emollient (white soft paraffin) over the entire epidermis, including denuded areas 1
• Apply nonadherent dressings to denuded dermis with secondary foam or burn dressings to collect exudate 1, 2
• Consider silver-containing products/dressings for sloughy areas only 1

Infection Prevention and Management

• Do NOT use prophylactic antibiotics as they increase skin colonization with resistant organisms, particularly Candida 1, 2
• Monitor for signs of infection rather than using prophylactic antibiotics 1, 2
• Obtain regular skin swabs for culture to detect predominant organisms 1, 2
• Institute targeted antimicrobial therapy only when clinical signs of infection are present 1, 2
• Fever from SJS/TEN itself complicates detection of secondary sepsis, requiring careful monitoring 1

Pain Management

• Provide adequate background simple analgesia with additional opioid analgesia for breakthrough pain 1

Mucosal Management

Ocular Care (Critical to Prevent Permanent Sequelae)

• Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews during the acute illness 3, 1, 2
• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 3, 1, 2
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 1, 2
• Daily examination for symblepharon formation with lysis of adhesions performed by an ophthalmologist to prevent permanent scarring 1
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 1, 2
• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 1
• Amniotic membrane transplantation (AMT) in the acute phase demonstrates significantly better visual outcomes compared to medical management alone 3

Oral Care

• Examine the mouth as part of initial assessment with daily oral review during acute illness 3, 1, 2
• Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness 3, 1, 2
• Clean the mouth daily with warm saline mouthwashes or an oral sponge, sweeping gently in the labial and buccal sulci to reduce risk of fibrotic scars 3, 1, 2
• Protect ulcerated mucosal surfaces with a mucoprotectant mouthwash three times daily (e.g., Gelclair) 3
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 3, 1, 2
• For inadequate pain control, use topical anesthetic preparation such as viscous lidocaine 2% (15 mL per application) or cocaine mouthwashes 2-5% three times daily for severe oral discomfort 3, 1, 2
• Use antiseptic oral rinse twice daily to reduce bacterial colonization: 1.5% hydrogen peroxide mouthwash (10 mL) or 0.2% chlorhexidine digluconate mouthwash (10 mL), diluting chlorhexidine by up to 50% to reduce soreness 3, 1, 2
• Take oral and lip swabs regularly if bacterial or candidal secondary infection is suspected 3
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 3

Urogenital Care

• Perform daily urogenital review during the acute illness 1, 2
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1, 2
• Use potent topical corticosteroid ointment once daily to involved, noneroded surfaces 1, 2
• Apply silicone dressings (e.g., Mepitel) to eroded areas to reduce pain and prevent adhesions 1, 2
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1
• Urinary catheterization when urogenital involvement causes dysuria or retention, or to monitor output 1

Systemic Immunomodulatory Therapy

The evidence for systemic therapy remains controversial, but based on available data:

• 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, 2
• Systemic corticosteroids, particularly early IV methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset 1, 2
• A large US multicenter study found the lowest standardized mortality ratio (0.52) among patients receiving both corticosteroids and intravenous immunoglobulin 4
• Overall mortality in this cohort was significantly lower than predicted (14.7% observed vs 21% expected), with the lowest mortality in those receiving combination therapy 4

Note: While systemic corticosteroids remain one of the most common treatment options, their efficacy remains uncertain in the literature 5. However, there is increasing evidence that cyclosporine and TNF-α inhibitors decrease mortality 5.

Airway Management

• Respiratory symptoms and hypoxemia on admission should prompt early discussion with an intensivist and rapid transfer to an ICU or burn center 2
• Fiberoptic bronchoscopy should be undertaken to assess airway involvement 2

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 1
• Manage pediatric patients in age-appropriate specialist units with pediatric intensivists and skin loss specialists 1
• High-risk children need quicker transfer to specialized care 1

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 or amulet 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
• Organize dermatology outpatient clinic appointment, and if required, ophthalmology outpatient appointment within a few weeks of discharge 2
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 1
• Consider referral to support groups such as SJS Awareness U.K. 1

Common Pitfalls to Avoid

• Delayed recognition and discontinuation of the culprit medication significantly increases mortality risk 1, 2
• Indiscriminate use of prophylactic antibiotics increases skin colonization with resistant organisms 1, 2
• Overaggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema 1, 2
• Failure to involve ophthalmology within 24 hours leads to permanent ocular sequelae 1, 2
• Continued use of the culprit medication worsens the condition and increases mortality 1

Quality Metrics for Audit

• SCORTEN performed on admission 1
• Ophthalmology consultation within 24 hours with daily assessments 1
• GP notification at discharge with drug avoidance counseling and MedicAlert request 1