What are the guidelines for managing Stevens-Johnson Syndrome (SJS)?

Management Guidelines for Stevens-Johnson Syndrome (SJS)

Immediate Life-Saving Actions

Immediately discontinue all suspected culprit drugs and transfer the patient to a specialized burn unit or ICU with multidisciplinary expertise in 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 (scores range 0-7, with higher scores indicating greater mortality) 1, 2, 3
• Document all medications taken in the previous 2 months, including over-the-counter and herbal products, with exact start dates 2, 3
• Obtain skin biopsy from lesional skin adjacent to a blister for histopathology showing confluent epidermal necrosis with subepidermal vesicle formation 2
• Early transfer to specialized centers reduces mortality; delays adversely affect outcomes 1, 2

Essential Supportive Care Framework

Wound and Skin Management

• Handle skin with extreme gentleness to minimize shearing forces that cause further epidermal detachment 1, 2
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 1, 2
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis every 4 hours, including denuded areas 1, 2
• Leave detached epidermis in situ to act as a biological dressing 1
• Apply nonadherent dressings (such as Mepitel or Telfa) to denuded dermis with secondary foam or burn dressings to collect exudate 1, 2

Fluid and Nutritional Management

• Establish adequate intravenous fluid resuscitation guided by urine output, avoiding overaggressive replacement that causes pulmonary, cutaneous, and intestinal edema 1, 2
• Provide continuous enteral nutrition throughout the acute phase: 20-25 kcal/kg daily during the catabolic phase and 25-30 kcal/kg during recovery 1, 3
• Use nasogastric feeding when oral intake is precluded by buccal mucositis 1

Environmental and General Measures

• Barrier nurse in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 1, 2
• Administer adequate analgesia using intravenous opioid infusions for those not tolerating oral medication, with patient-controlled analgesia where appropriate 2
• Provide low molecular weight heparin as prophylactic anticoagulation against venous thromboembolism in immobile patients 3

Critical Mucosal Management

Ophthalmologic Care (Most Disabling Complication)

Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews throughout the acute illness to prevent permanent visual impairment. 1, 2, 3

• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 1, 2
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 1
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 1
• Consider topical corticosteroids for ocular inflammation under ophthalmologist supervision 1
• Consider amniotic membrane transplantation in the acute phase, which demonstrates significantly better visual outcomes compared to medical management alone 1

Oral Care

• Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness 1
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 1
• Apply antiseptic oral rinse twice daily to reduce bacterial colonization 1
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 1

Urogenital Care

• Perform regular examination of urogenital tract during acute illness 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

Administer IV methylprednisolone 0.5-1 mg/kg (or equivalent) if started within 72 hours of onset, converting to oral corticosteroids on response with tapering over at least 4 weeks. 3, 4, 5

• Methylprednisolone is FDA-approved for severe erythema multiforme (Stevens-Johnson syndrome) 4
• Early corticosteroid therapy (within 72 hours) shows benefit in multiple studies 6, 1, 5
• Alternative option: Cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month, has shown benefit with reduced mortality compared to predicted rates 1, 3, 5
• The evidence for intravenous immunoglobulin (IVIg) remains equivocal and did not reach statistical significance in available studies 6

Infection Management (Leading Cause of Death)

Do NOT administer prophylactic systemic antibiotics as this increases skin colonization, particularly with Candida albicans and resistant organisms. 1, 2, 3

• Monitor carefully for clinical signs of infection (confusion, hypotension, reduced urine output, reduced oxygen saturation) 2
• Obtain regular skin swabs for bacterial and candidal culture from lesional skin, particularly sloughy areas 2
• Institute targeted antimicrobial therapy ONLY when clinical signs of infection are present 1, 2
• Watch for monoculture of organisms on culture swabs from multiple sites, indicating increased likelihood of invasive infection 2

Multidisciplinary Team Coordination

• Coordinate care through a team led by a dermatologist or plastic surgeon with expertise in skin failure 2, 3
• Include intensive care physicians, ophthalmologists, and specialist skincare nurses as core team members 1, 2
• Involve additional specialists based on organ involvement: respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, and pharmacy 2

Discharge Planning and Long-Term Follow-Up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 6, 1, 3
• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug 6, 1
• Document drug allergy in the patient's medical records and inform all healthcare providers involved in their care 6, 3
• Report the adverse drug reaction to pharmacovigilance authorities (MHRA in the U.K.) 6
• Arrange dermatology outpatient follow-up within weeks of discharge 6, 1
• Schedule ophthalmology follow-up to monitor for chronic ocular complications, which can develop weeks to months after the acute episode 6, 1
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 6, 1
• Consider referral to support groups such as SJS Awareness U.K. 6, 1

Special Considerations for Pediatric Patients

• Infection causes up to 50% of pediatric SJS/TEN cases; test for infective triggers (particularly Mycoplasma) and consult infectious disease team in all pediatric cases 1
• Manage pediatric patients in age-appropriate specialist units with pediatric intensivists and skin loss specialists 1

Critical Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality risk 1, 2, 3
• Failure to involve ophthalmology within 24 hours leads to permanent visual impairment 1, 2, 3
• Indiscriminate use of prophylactic antibiotics increases resistant organism colonization 1, 2, 3
• Overaggressive fluid resuscitation causes pulmonary, cutaneous, and intestinal edema 1, 2
• Continued use of the culprit medication worsens the condition and increases mortality 1
• Neglecting daily ocular examination and lysis of adhesions results in permanent ocular sequelae 6, 1

Quality Metrics for Audit

• SCORTEN performed on admission 6, 1
• Ophthalmology consultation within 24 hours with daily assessments 6, 1
• GP notification at discharge with drug avoidance counseling and MedicAlert request 6, 1