What is the treatment for Stevens-Johnson Syndrome (SJS)?

Treatment of Stevens-Johnson Syndrome (SJS)

The most critical first step in treating Stevens-Johnson Syndrome is immediate discontinuation of any potential culprit drug, followed by prompt 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. 1, 2

Initial Management

• Immediately discontinue any potential culprit medication as the first and most critical intervention 1
• Calculate SCORTEN within the first 24 hours of admission to predict mortality risk 3, 2
• Transfer patients with >10% body surface area epidermal detachment to a specialized burn unit or ICU with experience treating SJS/TEN 3, 2
• Place patient in a temperature-controlled room (25-28°C) on a pressure-relieving mattress with barrier nursing 3, 1

Supportive Care

Skin Management

• Handle skin carefully to minimize shearing forces and prevent further epidermal detachment 3, 1
• Apply bland emollients to support barrier function and reduce transcutaneous water loss 3, 1
• Use appropriate non-adherent dressings (such as Mepitel™ or Telfa™) on denuded dermis to reduce fluid and protein loss, limit microbial colonization, and control pain 3, 1
• Take regular swabs for bacterial and candidal culture from lesional skin, particularly sloughy areas 1, 2

Fluid Management

• Carefully monitor fluid balance with regular assessment of vital signs and urine output 1, 2
• Establish adequate intravenous fluid replacement while avoiding fluid overload that can lead to pulmonary, cutaneous, and intestinal edema 1, 2

Infection Prevention

• Monitor for signs of systemic infection rather than using prophylactic antibiotics, which may increase skin colonization with resistant organisms 3, 1
• Only institute antimicrobial therapy when clinical signs of infection are present 3, 1

Mucosal Management

Ocular Care

• Arrange ophthalmology consultation within 24 hours of diagnosis for all patients 1, 2
• Apply frequent lubrication with preservative-free artificial tears and lubricating ointments 1
• Perform daily examination for symblepharon formation with lysis of adhesions by an ophthalmologist to prevent permanent scarring 1
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 1
• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 1

Oral Care

• Use anti-inflammatory oral rinses containing benzydamine hydrochloride every 3 hours, particularly before eating 1
• Apply antiseptic oral rinses twice daily 1
• Consider topical anesthetics such as viscous lidocaine 2% for severe oral discomfort 1
• Monitor for and treat secondary infections with appropriate antifungals or antivirals if needed 1

Urogenital Care

• Consider urinary catheterization when urogenital involvement causes dysuria or retention 1
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1
• Consider vaginal dilators or tampons wrapped in non-adherent dressings to prevent vaginal synechiae formation 1

Systemic Therapy Options

• Systemic corticosteroids may be beneficial if started early (preferably within 72 hours), using moderate to high doses (prednisolone 1-2 mg/kg/day or equivalent) tapered rapidly within 7-10 days 3, 1, 4
• Cyclosporine (3-5 mg/kg/day for 10-14 days) may be used either alone or in combination with corticosteroids 3, 4
• Etanercept (TNF-alpha inhibitor) may reduce mortality compared to corticosteroids alone, though evidence is limited 5, 6
• Intravenous immunoglobulin (IVIG) has been used but with uncertain benefit based on current evidence 5, 7

Multidisciplinary Approach

• Coordinate care through a multidisciplinary team including dermatology, intensive care, ophthalmology, and specialist skincare nursing 3, 1
• Consider additional input from respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, and pharmacy as needed 3, 1

Common Pitfalls to Avoid

• Delayed recognition and treatment significantly increases mortality risk 1, 2
• Indiscriminate use of prophylactic antibiotics may increase skin colonization with resistant organisms 3, 1
• Overaggressive fluid resuscitation can lead to pulmonary, cutaneous, and intestinal edema 1, 2
• Failure to involve ophthalmology early can lead to permanent visual impairment 1, 2
• Continued use of the culprit medication will worsen the condition and increase mortality 1, 2