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

Management of Stevens-Johnson Syndrome (SJS)

Immediate Actions

Immediately discontinue all suspected culprit drugs and transfer the patient to a specialized burn unit or ICU with multidisciplinary expertise, particularly when body surface area involvement exceeds 10%. 1, 2, 3

• 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
• Early transfer to specialized centers is critical—delays in recognition and transfer significantly increase mortality risk 1, 2, 3
• Obtain skin biopsy showing confluent epidermal necrosis with subepidermal vesicle formation to confirm diagnosis 3
• Document all medications taken in the previous 2 months, including over-the-counter and herbal products, with exact start dates 1, 3

Systemic Immunomodulatory Therapy

Administer IV methylprednisolone 0.5-1 mg/kg if started within 72 hours of onset, converting to oral corticosteroids on response with tapering over at least 4 weeks. 1, 2, 4

• The FDA label specifically indicates methylprednisolone for severe erythema multiforme (Stevens-Johnson syndrome) 4
• Alternative option: Cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month, which has shown reduced mortality compared to predicted rates 1, 2
• Both therapies are most effective when initiated within 72 hours of symptom onset 1, 2, 5

Essential Supportive Care

Skin Management

• Handle skin with extreme gentleness to minimize shearing forces that cause further epidermal detachment 1, 2, 3
• 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, 2
• Apply nonadherent dressings (Mepitel™ or Telfa™) to denuded dermis with secondary foam or burn dressings to collect exudate 2, 3

Environmental Control

• Barrier nurse in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 2, 3
• Control humidity in the patient's room 3

Fluid Management

• Provide careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload 2, 3
• Monitor fluid balance with regular assessment of vital signs, urine output, and electrolytes 2, 3
• Avoid overaggressive fluid resuscitation, which causes pulmonary, cutaneous, and intestinal edema 1, 2, 3

Nutritional Support

• Deliver continuous enteral nutrition throughout the acute phase: 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

• Provide adequate background simple analgesia with additional opioid analgesia for breakthrough pain 2
• Use validated pain assessment tools at least once daily 3
• Consider patient-controlled analgesia or sedation/general anesthesia for dressing changes 3

Critical Mucosal Management

Ophthalmologic Care

Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews throughout the acute illness—failure to do so leads to 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, 2
• Consider topical corticosteroids for ocular inflammation under ophthalmologist supervision 1, 2
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 2
• Consider amniotic membrane transplantation in the acute phase for significantly better visual outcomes 2

Oral Care

• Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness 2
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 2
• Apply antiseptic oral rinse twice daily 2
• Use 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
• Consider 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

Infection Management

Do NOT administer prophylactic systemic antibiotics—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) 1, 3
• Obtain regular skin swabs for bacterial and candidal culture from lesional skin 1, 3
• Institute targeted antimicrobial therapy ONLY when clinical signs of infection are present 1, 2, 3
• Watch for monoculture of organisms on culture swabs from multiple sites, indicating increased likelihood of invasive infection 1, 3
• Septicemia is the most common cause of death in SJS/TEN 3, 6

Multidisciplinary Team Coordination

• Coordinate care through a team led by a dermatologist or plastic surgeon with expertise in skin failure 1, 2, 3
• Core team members include intensive care physicians, ophthalmologists, and specialist skincare nurses 1, 2, 3
• Additional specialists may include respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, and pharmacy 2, 3

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, 3
• Manage pediatric patients in age-appropriate specialist units with pediatric intensivists and skin loss specialists 1, 3

Discharge Planning and Long-Term Follow-Up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 1, 2, 3
• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug 1, 2, 3
• Document drug allergy in the patient's medical records and inform all healthcare providers involved in their care 1, 2, 3
• Report the adverse drug reaction to pharmacovigilance authorities 1, 2, 3
• Arrange follow-up with dermatology and ophthalmology within weeks of discharge 3
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 2

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, 3
• Continued use of the culprit medication will worsen the condition and increase mortality 2