What is the management of Stevens-Johnson Syndrome (SJS)?

Management of Stevens-Johnson Syndrome

Immediate Life-Saving Actions

Stop all suspected culprit drugs immediately and transfer the patient to a specialized burn unit or ICU with experience managing SJS/TEN, particularly when body surface area involvement exceeds 10%. 1, 2

• Calculate SCORTEN within the first 24 hours of admission to predict mortality risk and guide intensity of care 2, 3
• Arrange immediate ophthalmology consultation within 24 hours of diagnosis—failure to do so can result in permanent visual impairment 2, 3
• Transfer without delay reduces mortality; delays in specialized care adversely affect outcomes 2

Multidisciplinary Team Approach

Coordinate care through a multidisciplinary team including dermatology, intensive care, burn surgery, ophthalmology, and specialist skincare nursing. 2, 3

• Additional specialists may include respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, and physiotherapy as needed 3
• For pediatric patients, manage in age-appropriate specialist units with pediatric intensivists 2

Supportive Care Framework

Environmental and General Measures

• Place patient in a temperature-controlled room (25-28°C) on a pressure-relieving mattress with barrier nursing techniques 2, 3
• Monitor vital signs, urine output, and electrolytes regularly 2

Fluid Management

• Establish adequate intravenous fluid replacement guided by urine output and other end-point measurements 3
• Avoid overaggressive fluid resuscitation which may cause pulmonary, cutaneous, and intestinal edema 2, 3
• Consider using the formula: body weight/% BSA epidermal detachment to determine replacement volumes 3

Wound Care

• Handle skin carefully to minimize shearing forces and prevent further epidermal detachment 2, 3
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 2
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis, including denuded areas 2
• Leave detached epidermis in situ to act as a biological dressing 2
• Decompress blisters by piercing and expression or aspiration of fluid 2
• Apply nonadherent dressings (such as Mepitel™ or Telfa™) to denuded dermis with secondary foam or burn dressings to collect exudate 2, 3
• Consider silver-containing products/dressings for sloughy areas only 2

Nutrition

• Deliver continuous enteral nutrition throughout the acute phase, providing 20-25 kcal/kg daily during the catabolic phase and 25-30 kcal/kg during recovery 2
• Consider nasogastric feeding when oral intake is precluded by buccal mucositis 2

Pain Management

• Use validated pain assessment tools at least once daily 3
• Provide adequate background simple analgesia with additional opioid analgesia for breakthrough pain 2
• Administer intravenous opioid infusions for those not tolerating oral medication 3
• Consider patient-controlled analgesia where appropriate 3
• Consider sedation or general anesthesia for patient handling, repositioning, and dressing changes 3

Infection Prevention and Management

Do not administer prophylactic systemic antibiotics as this increases skin colonization with resistant organisms, particularly Candida albicans. 2, 3

• Monitor for signs of systemic infection (confusion, hypotension, reduced urine output, reduced oxygen saturation) 3
• Take regular skin swabs for bacterial and candidal culture from lesional skin, particularly sloughy areas 2, 3
• Institute targeted antimicrobial therapy only when clinical signs of infection are present 2, 3
• Watch for monoculture of organisms on culture swabs from multiple sites, which indicates increased likelihood of invasive infection 3
• Fever from SJS/TEN itself complicates detection of secondary sepsis, requiring careful monitoring 2

Mucosal Management

Ocular Care (Critical Priority)

Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews during the acute illness—this is essential to prevent permanent ocular sequelae. 2

• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 2
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 2
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 2
• Consider topical corticosteroids for ocular inflammation under ophthalmologist supervision 2
• Consider amniotic membrane transplantation (AMT) in the acute phase, which demonstrates significantly better visual outcomes compared to medical management alone 2

Oral Care

• Examine the mouth as part of initial assessment with daily oral review during acute illness 2
• Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness to reduce risk of fibrotic scars 2
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 2
• Use antiseptic oral rinse twice daily to reduce bacterial colonization 2
• Apply topical anesthetics such as viscous lidocaine 2% or cocaine mouthwashes 2-5% for severe oral discomfort 2
• 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 2
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 2
• Use urinary catheterization when urogenital involvement causes dysuria or retention, or to monitor output 2
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 2

Systemic Immunomodulatory Therapy

Cyclosporine (3 mg/kg daily for 10 days, tapered over 1 month) is recommended as it has shown benefit in multiple studies with reduced mortality compared to predicted rates. 2

• Systemic corticosteroids, particularly early methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset 2
• The evidence for immunomodulating agents remains limited, with high-quality studies still lacking 4

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 2
• Recurrence is more common in children (up to 18% of cases), perhaps because the precipitant is usually infection rather than drugs 1
• Mortality appears lower in children than adults, making management of long-term sequelae particularly important 1

Discharge Planning and Follow-Up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 2
• Encourage patients to wear a MedicAlert bracelet or amulet bearing the name of the culprit drug 2
• Document the drug allergy in the patient's medical records and inform all healthcare providers involved in their care 2
• Report the adverse drug reaction to pharmacovigilance authorities 2, 3
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 2
• Arrange follow-up with dermatology and ophthalmology within weeks of discharge 3
• Consider referral to support groups such as SJS Awareness U.K. 2

Critical Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality risk 2, 5
• Indiscriminate use of prophylactic antibiotics increases skin colonization with resistant organisms 2, 3
• Overaggressive fluid resuscitation causes pulmonary, cutaneous, and intestinal edema 2, 3
• Failure to involve ophthalmology early leads to permanent visual impairment 2, 3
• Continued use of the culprit medication worsens the condition and increases mortality 2
• Neglecting eye care may result in permanent visual impairment 3