What is the treatment for Stevens-Johnson syndrome?

Treatment of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit drugs and transfer patients with >10% body surface area involvement to a specialized burn unit or ICU without delay, as this is the most critical intervention to reduce mortality. 1, 2

Immediate Actions

• Stop all potential causative medications immediately upon suspicion of SJS, as continued exposure dramatically worsens outcomes and increases mortality 2, 3
• Calculate SCORTEN within 24 hours of admission using 7 clinical parameters (age, BSA involvement, heart rate, serum glucose, bicarbonate, BUN, malignancy) to predict mortality risk: score 0-1 = 3.2% mortality, score 5+ = 90% mortality 2, 3
• Transfer to specialized centers (burn unit or ICU) is mandatory for patients with >10% BSA epidermal detachment, as delayed transfer significantly increases mortality 1, 2, 4
• Barrier nurse in a temperature-controlled room (25-28°C) on a pressure-relieving mattress to minimize further skin damage 2, 4

Supportive Care Framework

Fluid Management

• Use the formula: body weight (kg) × % BSA epidermal detachment = mL/hour for fluid replacement, which is significantly less aggressive than burn formulas (Parkland) 3
• Monitor vital signs, urine output, and electrolytes regularly to prevent end-organ hypoperfusion while avoiding fluid overload that causes pulmonary, cutaneous, and intestinal edema 1, 2

Skin and Wound Care

• Leave detached epidermis in situ to act as a biological dressing 2, 3
• Cleanse wounds regularly by gently irrigating with warmed sterile water, saline, or chlorhexidine (1:5000) 2
• Apply greasy emollients (50% white soft paraffin with 50% liquid paraffin) over entire epidermis every 2-4 hours, including denuded areas, to support barrier function and reduce transcutaneous water loss 1, 2
• Use nonadherent dressings on denuded dermis with secondary foam or burn dressings to collect exudate 2
• Minimize shearing forces when handling skin to prevent further epidermal detachment 2

Infection Prevention

• Do NOT use prophylactic antibiotics, as they increase skin colonization with resistant organisms, particularly Candida 1, 2, 3
• Obtain regular skin swabs for culture to detect predominant organisms 2
• Institute targeted antimicrobial therapy only when clinical signs of infection appear (confusion, hypotension, reduced urine output, oxygen desaturation, increased skin pain) 2, 3

Nutrition

• Provide continuous enteral nutrition throughout the acute phase, delivering 20-25 kcal/kg daily during the early catabolic phase and 25-30 kcal/kg during recovery 1, 2
• Use nasogastric feeding with silicone tube when oral intake is precluded by buccal mucositis 1, 2

Pain Management

• Provide adequate background simple analgesia to ensure comfort at rest using the WHO analgesic ladder 1, 2
• Add opiates (morphine) as required via enteral route, patient-controlled analgesia, or infusion for moderate-to-severe pain 1
• Monitor level of consciousness, respiratory rate, and oxygen saturation carefully when using opiate infusions 1

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients to prevent venous thromboembolism 1
• Consider proton pump inhibitor during acute phase when enteral nutrition cannot be established to protect against upper gastrointestinal stress ulceration 1
• Consider recombinant human G-CSF for neutropenic patients to reduce risk of life-threatening sepsis 1

Mucosal Management

Ophthalmologic Care (Critical Priority)

• Obtain ophthalmology consultation within 24 hours of diagnosis, as 74% of patients develop acute ocular involvement and 50-63% develop chronic complications (severe dry eyes, trichiasis, corneal scarring, vision loss) 1, 2, 3, 4
• Perform daily ophthalmologic examinations throughout the acute phase by an ophthalmologist or ophthalmically trained nurse 2, 4
• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 2, 4
• Perform daily ocular hygiene to remove inflammatory debris and break down conjunctival adhesions to prevent symblepharon formation 2
• 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, 2
• Consider amniotic membrane transplantation (AMT) in the 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 2
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 1, 2
• Use antiseptic oral rinse twice daily to reduce bacterial colonization 1, 2
• Apply topical anesthetics such as viscous lidocaine 2% or cocaine mouthwashes 2-5% for severe oral discomfort 1
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 2

Urogenital Care

• Perform regular examination of urogenital tract during acute illness 1
• Use urinary catheterization when urogenital involvement causes dysuria or retention, or to monitor output 1
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1

Systemic Immunomodulatory Therapy

The evidence for systemic immunomodulation remains controversial, with no definitive consensus, but early intervention within 72 hours appears beneficial 5, 6:

First-Line Options

• 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 2, 7, 6
• IV methylprednisolone (0.5-1 mg/kg for Grade 3, or 1-2 mg/kg for Grade 4) may be beneficial if started within 72 hours of onset, tapering when toxicity resolves 2, 3, 7

Second-Line Options

• High-dose IVIG (2-3 g/kg over 3-5 days, typically 1 g/kg/day for 3 days) may be added in severe or steroid-unresponsive cases 3
• TNF-α inhibitors show increasing evidence of decreased mortality 6

Note: High-quality randomized controlled trials assessing efficacy of immunomodulating agents are still lacking 5, 6

Multidisciplinary Team Requirements

Essential team members include 2, 4:

• Dermatology
• Intensive care/burn surgery
• Ophthalmology (mandatory)
• Specialist skincare nursing
• Additional consultants based on organ involvement: otolaryngology, urology/gynecology, infectious disease

Discharge Planning and Follow-up

• Provide written information about culprit drug(s) to avoid and any potentially cross-reactive medications 2, 4
• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug 2, 4
• Document drug allergy in patient's medical records and inform all healthcare providers involved in their care 2, 4
• Report adverse drug reaction to pharmacovigilance authorities 2
• Inform patients about potential fatigue and lethargy for several weeks following discharge and need for convalescence 2
• Arrange long-term multidisciplinary follow-up for cutaneous, ocular, and psychological sequelae 5

Critical Pitfalls to Avoid

• Delayed transfer to specialized care is associated with significantly increased mortality 2, 3, 4
• Indiscriminate prophylactic antibiotics increase Candida colonization and promote resistant organisms 1, 2, 3
• Overaggressive fluid resuscitation using burn formulas leads to pulmonary, cutaneous, and intestinal edema 1, 3
• Failure to involve ophthalmology early leads to permanent ocular sequelae in the majority of patients 1, 2, 3
• Continued use of culprit medication dramatically worsens condition and increases mortality 2, 3