What is the treatment for Steven's Johnson Syndrome (SJS)?

Treatment of Stevens-Johnson Syndrome (SJS)

The treatment of Stevens-Johnson Syndrome requires immediate discontinuation of the suspected causative agent, admission to a burn unit or ICU for patients with >10% body surface area involvement, and comprehensive supportive care including fluid management, wound care, and management of mucosal complications. 1

Initial Management

• Immediate discontinuation of any potential culprit drug is the first and most critical step in SJS management 1
• Assess severity using SCORTEN (severity-of-illness score for toxic epidermal necrolysis) to predict mortality risk 1
• Transfer to specialized care center with experience managing SJS/TEN, preferably a burn unit or ICU for patients with >10% body surface area (BSA) epidermal detachment 1
• Barrier nursing in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 1

Supportive Care

Fluid Management

• Careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload that can lead to pulmonary, cutaneous, and intestinal edema 1
• Monitor fluid balance with regular assessment of vital signs, urine output, and electrolytes 1

Wound Care

• Gentle skin handling to minimize shearing forces and prevent further epidermal detachment 1
• Frequent application of bland emollients to support barrier function and reduce transcutaneous water loss 1
• Appropriate dressings on exposed dermis to reduce fluid and protein loss, limit microbial colonization, control pain, and accelerate re-epithelialization 1
• Consider petrolatum emollients or dimethicone as alternatives for skin protection 1
• High-strength topical corticosteroids may be used on affected skin areas 1

Infection Prevention and Management

• Monitor for signs of infection rather than using prophylactic antibiotics, which may increase skin colonization 1
• Regular skin swabs for culture to detect predominant organisms 1
• Targeted antimicrobial therapy only when clinical signs of infection are present 1

Mucosal Management

Ocular Care

• Early ophthalmology consultation for all patients with ocular involvement 1
• Frequent lubrication with preservative-free artificial tears and lubricating ointments 1
• Daily examination for symblepharon (adhesions) formation 1
• Lysis of adhesions performed by an ophthalmologist to prevent permanent scarring 1
• Topical antibiotics (e.g., quinolones like moxifloxacin or levofloxacin) when corneal fluorescein staining or ulceration is present 1
• Topical corticosteroid drops (e.g., nonpreserved dexamethasone 0.1%) under ophthalmologist supervision may reduce ocular surface damage 1

Oral Care

• Anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 1
• Antiseptic oral rinse twice daily (e.g., 1.5% hydrogen peroxide or 0.2% chlorhexidine digluconate mouthwash) 1
• Topical anesthetics such as viscous lidocaine 2% or cocaine mouthwashes 2-5% for severe oral discomfort 1
• Monitor for and treat secondary infections with appropriate antifungals (nystatin or miconazole) or antivirals if HSV is suspected 1
• Topical corticosteroids may be applied to oral mucosa (e.g., betamethasone sodium phosphate or clobetasol propionate 0.05%) 1

Urogenital Care

• Urinary catheterization when urogenital involvement causes dysuria or retention, or to monitor output 1
• Regular examination of urogenital tract during acute illness 1
• White soft paraffin ointment applied to urogenital skin and mucosae every 4 hours 1
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1

Systemic Therapy Options

• Systemic corticosteroids: IV methylprednisolone (0.5-1 mg/kg/day for moderate cases, 1-2 mg/kg/day for severe cases) with rapid tapering over 7-10 days may be beneficial if started early (preferably within 72 hours) 1, 2
• Cyclosporine (3-5 mg/kg/day for 10-14 days) may be used alone or in combination with corticosteroids 2
• Intravenous immunoglobulin (IVIG) or cyclosporine may be considered in severe or steroid-unresponsive cases 1, 3

Special Considerations

• Multidisciplinary approach involving dermatology, intensive care, ophthalmology, and specialist skincare nursing is essential 1
• Additional specialists may be needed based on organ involvement (respiratory, gastroenterology, gynecology, urology, oral medicine) 1
• Monitor for complications affecting ocular, cutaneous, renal, pulmonary, and hepatic systems 4
• Long-term follow-up is necessary to address potential sequelae, particularly ocular and urogenital scarring 5

Common Pitfalls to Avoid

• Delayed recognition and treatment significantly increases mortality risk 1
• Indiscriminate use of prophylactic antibiotics may increase skin colonization with resistant organisms 1
• Overaggressive fluid resuscitation can lead to pulmonary, cutaneous, and intestinal edema 1
• Failure to involve appropriate specialists early, particularly ophthalmology, can lead to permanent sequelae 1
• Continued use of the culprit medication will worsen the condition and increase mortality 1