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

Treatment of Stevens-Johnson Syndrome

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

Initial Assessment and Triage

• Calculate SCORTEN on admission to predict mortality risk (ranging from 1% with 0 parameters to 99% with 7 parameters) and guide intensity of care 1, 2
• Transfer without delay to centers with experience managing SJS/TEN, as early transfer reduces mortality while delays adversely affect outcomes 2
• The multidisciplinary team must include dermatology, intensive care, burn surgery, ophthalmology, and specialist skincare nursing 2, 3

Supportive Care Framework

Skin and Wound Management

• Use barrier nursing in a temperature-controlled room (25-28°C) on a pressure-relieving mattress to minimize shearing forces that cause further epidermal detachment 1, 2
• Leave detached epidermis in situ to act as a biological dressing, and decompress blisters by piercing and expressing fluid 2
• Apply bland emollients frequently (white soft paraffin or similar) to the entire skin surface to support barrier function, reduce transcutaneous water loss, and encourage re-epithelialization 1, 2
• Cover denuded dermis with appropriate nonadherent dressings (such as Mepitel) with secondary foam or burn dressings to reduce fluid and protein loss, limit microbial colonization, control pain, and accelerate healing 1, 2
• Consider silver-containing dressings only for sloughy areas 2

Fluid and Nutritional Management

• Provide careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload that can lead to pulmonary, cutaneous, and intestinal edema 1, 2
• Monitor fluid balance with regular assessment of vital signs, urine output, and electrolytes 2
• Deliver continuous enteral nutrition providing 20-25 kcal/kg daily during the catabolic phase and 25-30 kcal/kg during recovery; consider nasogastric feeding when oral intake is precluded by buccal mucositis 2

Infection Prevention and Management

• Do NOT use prophylactic antibiotics, as indiscriminate administration increases skin colonization with resistant organisms, particularly Candida albicans 1, 2
• Obtain regular skin swabs for culture to detect predominant organisms 1, 2
• Institute targeted antimicrobial therapy only when clinical signs of infection are present (confusion, hypotension, reduced urine output, reduced oxygen saturation, increased skin pain, rising C-reactive protein, neutrophilia, or monoculture on swabs) 1, 2
• Monitor carefully as fever from SJS/TEN itself complicates detection of secondary sepsis 1, 2

Pain Management

• Provide adequate background simple analgesia with additional opioid analgesia for breakthrough pain 2

Mucosal Management

Ocular Care (Critical for Preventing Blindness)

• Arrange ophthalmology consultation within 24 hours of diagnosis with daily examinations throughout the acute phase 2, 3
• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 2, 3
• 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 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 2
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 1, 2
• Use antiseptic oral rinse twice daily to reduce bacterial colonization 1, 2
• Apply topical corticosteroids (betamethasone sodium phosphate 0.5 mg in 10 mL water as a 3-minute rinse-and-spit preparation four times daily, or clobetasol propionate 0.05% mixed with Orabase applied directly to affected areas) 1
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week 2
• Consider topical anesthetics such as viscous lidocaine 2% for severe oral discomfort 2

Urogenital Care

• Examine the urogenital tract as part of initial assessment with daily documented review during acute illness 1
• Catheterize all patients to prevent urethral strictures and monitor urine output 1, 2
• Apply white soft paraffin ointment to urogenital skin and mucosae immediately and every 4 hours throughout the acute illness 1, 2
• In women, arrange early assessment by a vulval specialist for consideration of dilators to prevent vaginal synechiae 1
• Use Mepitel dressings on eroded areas in the vulva and vagina to reduce pain and prevent adhesions; insert a dilator or tampon wrapped in Mepitel into the vagina to prevent synechiae formation 1, 2
• Apply potent topical corticosteroid ointment once daily to involved, noneroded urogenital surfaces 1
• Check uncircumcised male patients for preputial retractability 1

Systemic Immunomodulatory Therapy

• Ciclosporin (3 mg/kg daily for 10 days, tapered over 1 month) has shown benefit in multiple studies with reduced mortality compared to predicted rates 2, 4, 5
• Systemic corticosteroids, particularly IV methylprednisolone pulse therapy, may be beneficial if started early, preferably within 72 hours of onset 2, 3, 4
• The efficacy of intravenous immunoglobulins remains uncertain and requires further study 6, 4, 5
• TNF-α inhibitors show increasing evidence of decreasing mortality but require further multicenter trials 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
• Manage pediatric patients in age-appropriate specialist units with pediatric intensivists and skin loss specialists 2

Discharge Planning and Follow-up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 2, 3
• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug 2, 3
• Document the drug allergy in the patient's medical records and inform all healthcare providers involved in their care 2, 3
• Report the adverse drug reaction to pharmacovigilance authorities 2
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 2
• Consider referral to support groups such as SJS Awareness U.K. 2
• Arrange long-term follow-up for monitoring of ocular sequelae (chronic dry eye, symblepharon, corneal scarring), cutaneous sequelae (pigmentation changes, scarring), and urogenital sequelae (urethral strictures, phimosis, vaginal synechiae, sexual dysfunction) 3

Common Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality 2
• Continued use of the culprit medication will worsen the condition and increase mortality 2
• Indiscriminate prophylactic antibiotics increase resistant organism colonization 1, 2
• Overaggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema 2
• Failure to involve ophthalmology early leads to permanent ocular sequelae including blindness 2, 3