Treatment of Stevens-Johnson Syndrome
Supportive care is the cornerstone of management for Stevens-Johnson syndrome (SJS), with immediate discontinuation of any potential culprit drug as the first and most critical step. 1
Initial Management
- Immediately discontinue any suspected causative medication 1
- Assess severity using SCORTEN to predict mortality risk 2
- Transfer to a specialized care center with experience managing SJS/TEN, preferably a burn unit or ICU for patients with >10% body surface area epidermal detachment 2
- Barrier nursing in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 2
Multidisciplinary Team Approach
- Treatment should involve specialists from dermatology, intensive care, ophthalmology, and specialist skincare nursing 2
- Additional clinical input may be required from respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy and pharmacy based on organ involvement 2
Skin Management
- Gentle skin handling to minimize shearing forces and prevent further epidermal detachment 2
- Frequent application of bland emollients to support barrier function and reduce transcutaneous water loss 2
- Appropriate dressings on exposed dermis to reduce fluid and protein loss, limit microbial colonization, control pain, and accelerate re-epithelialization 2
- Conservative approach is generally preferred, but surgical debridement may be considered in cases of clinical deterioration, extension of epidermal detachment, local sepsis, or delayed healing 2
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 formation with lysis of adhesions to prevent permanent scarring 1
- Topical antibiotics when corneal fluorescein staining or ulceration is present 1
- Amniotic membrane transplant may be needed for severe eye disease 2
Oral Care
- Anti-inflammatory oral rinses containing benzydamine hydrochloride every 3 hours, particularly before eating 1
- Antiseptic oral rinses twice daily 1
- Topical anesthetics for severe oral discomfort 1
- Monitor for and treat secondary infections 1
Urogenital Care
- Urinary catheterization when urogenital involvement causes dysuria or retention 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 to prevent vaginal synechiae formation 1
Fluid and Nutritional Management
- Careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload 1
- Monitor fluid balance with regular assessment of vital signs, urine output, and electrolytes 1
- Maintain nutrition through appropriate feeding strategies 3
Infection Prevention and Management
- Monitor for signs of infection rather than using prophylactic antibiotics, which may increase skin colonization with resistant organisms 2
- Regular skin swabs for culture to detect predominant organisms 1
- Targeted antimicrobial therapy only when clinical signs of infection are present 2
Systemic Therapy Options
- Evidence for systemic therapies is limited and controversial 2
- Systemic corticosteroids may be beneficial if started early (within 72 hours), but evidence is conflicting 2, 1
- Cyclosporin has shown some benefit in adults according to meta-analysis data 2
- Intravenous immunoglobulin (IVIG) has been used but with inconsistent results 2
- TNF inhibitors (etanercept) may reduce mortality compared to corticosteroids, but evidence is limited 4
Psychological Support
- Constant psychological support for both patient and nursing staff is necessary 3
- Education and reassurance should be as much a part of the treatment process as drug therapy 3
Common Pitfalls to Avoid
- Delayed recognition and treatment significantly increases mortality risk 2
- Indiscriminate use of prophylactic antibiotics may increase skin colonization with resistant organisms 2
- 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