What is the treatment for Steven Johnson Syndrome (SJS)?

Treatment of Stevens-Johnson Syndrome

Immediate Life-Saving Actions

Immediately discontinue all suspected culprit medications upon first suspicion of SJS, as this single intervention most directly impacts survival. 1, 2, 3

• Calculate SCORTEN within the first 24 hours to predict mortality risk (scores 0-7 correlate with mortality from 1% to 99%). 1, 2, 3
• Transfer patients with >10% body surface area epidermal detachment to a specialized burn unit or ICU without delay—delayed transfer significantly increases mortality. 1, 2, 3
• Barrier-nurse patients in a temperature-controlled room (25-28°C) on a pressure-relieving mattress to prevent hypothermia and reduce infection risk. 1, 2

Supportive Care Framework

Fluid and Hemodynamic Management

• Establish adequate intravenous fluid replacement guided by urine output, avoiding overaggressive resuscitation that causes pulmonary, cutaneous, and intestinal edema. 1, 2, 3
• Monitor fluid balance with urinary catheterization when clinically indicated, checking vital signs, urine output, and electrolytes regularly. 1, 2
• In severely affected cases, use continuous invasive hemodynamic monitoring through central or arterial lines, measuring serum lactate, base deficit, and urea/electrolytes to detect tissue hypoperfusion. 1

Wound Care Protocol

• Handle skin with extreme care to minimize shearing forces that cause further epidermal detachment. 1, 2, 3
• Leave detached epidermis in situ to act as a biological dressing—do not debride intact blisters. 1, 2, 3
• Decompress blisters by piercing and expressing fluid without removing the overlying epidermis. 2
• Irrigate wounds gently using 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 every few hours. 2, 3
• Apply nonadherent dressings to denuded dermis with secondary foam or burn dressings to collect exudate. 2, 3
• Consider silver-containing dressings for sloughy areas only, changing every 3 days. 4
• Avoid adhesive dressings, ECG leads, blood pressure cuffs, and identification tags that traumatize skin—use soft silicone tapes when essential items must be secured. 1

Infection Prevention and Management

Do not use prophylactic systemic antibiotics—indiscriminate administration increases skin colonization with resistant organisms, particularly Candida. 1, 2, 3

• Take swabs for bacterial and candidal culture from three lesional areas on alternate days throughout the acute phase. 1, 2, 3
• Take viral swabs from eroded areas if HSV infection is suspected. 1
• Administer targeted antimicrobial therapy only when clinical signs of infection appear (fever, hemodynamic instability, rising C-reactive protein, neutrophilia, positive cultures). 1, 2, 3
• Change peripheral venous cannulas every 2-3 days through nonlesional skin if signs of sepsis or local infection develop. 1
• Change central lines every 5-7 days through nonlesional skin if signs of sepsis develop. 1

Nutrition Support

• Provide continuous enteral nutrition throughout the acute phase, either orally or via nasogastric/nasojejunal feeding when oral intake is precluded by buccal mucositis. 2, 3
• Deliver 20-25 kcal/kg daily during the catabolic phase. 2, 3
• Increase to 25-30 kcal/kg daily during the anabolic recovery phase. 2

Pain Management

• Use validated pain assessment tools at least once daily in conscious patients. 1, 3
• Provide adequate background simple analgesia (paracetamol, NSAIDs) to ensure comfort at rest. 2, 3
• Add opioid analgesia for breakthrough pain, delivered enterally, by patient-controlled analgesia (PCA), or via intravenous infusion. 1, 2, 3
• Consider sedation or general anesthesia for pain associated with patient handling, repositioning, and dressing changes. 1
• In severely affected cases, consider keeping the patient sedated and ventilated in ICU for the duration of the acute phase. 1

Mucosal Management

Ocular Care (Critical for Preventing Blindness)

Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews throughout the acute illness—50-63% develop late complications including severe dry eyes and trichiasis. 1, 2, 3, 5

• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness. 1, 2, 3
• Apply lubricating ointments regularly. 2
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions (lysis of adhesions prevents permanent scarring). 1, 2, 3
• Use topical antibiotics when corneal fluorescein staining or ulceration is present. 1, 2
• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage. 1, 2
• Consider amniotic membrane transplantation in the acute phase—demonstrates significantly better visual outcomes compared to medical management alone. 2

Oral Care

• Perform daily oral review during the acute illness. 2, 3
• Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness to reduce risk of fibrotic scars. 2, 3
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating. 1, 2, 3
• Use antiseptic oral rinse twice daily to reduce bacterial colonization. 1, 2
• Apply topical anesthetics such as viscous lidocaine 2% or cocaine mouthwashes 2-5% for severe oral discomfort. 1
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole. 2
• Consider topical corticosteroids applied to oral mucosa. 1

Urogenital Care

• Perform regular examination of urogenital tract during acute illness. 1
• Catheterize when urogenital involvement causes dysuria or retention, or to monitor urine output. 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

Cyclosporine 3 mg/kg daily for 10 days (tapered over 1 month) has shown the most consistent benefit with reduced mortality compared to predicted rates in multiple studies. 2, 3, 6

• If using corticosteroids, start early (preferably within 72 hours of onset) with IV methylprednisolone 0.5-1 mg/kg or oral prednisolone 1-2 mg/kg/day. 2, 3, 5
• Taper and discontinue steroids if initiated at an outside facility, as prolonged use increases infection risk. 4
• Intravenous immunoglobulin (IVIg) evidence is equivocal—pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75). 2
• A combination of IVIg and corticosteroids may be most effective for SJS/TEN overlap and TEN. 6
• Avoid thalidomide—it was associated with excess deaths in one randomized trial. 2

Airway and Respiratory Management

• Respiratory symptoms and hypoxemia on admission require urgent intensivist discussion and rapid ICU/burn center transfer. 2
• Perform fiberoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, investigate pneumonitis, and mechanically remove sloughed bronchial epithelium. 2
• Intubate and ventilate only in extreme circumstances due to complications including nosocomial pneumonia and fluid overload. 1

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients unless contraindicated. 2, 3
• Provide proton pump inhibitor to protect against upper gastrointestinal stress ulceration if enteral nutrition cannot be established. 2, 3
• Consider recombinant human G-CSF for neutropenic patients. 2
• Consider faecal management system in immobile patients with diarrhea to prevent fecal soiling of wounds. 1

Multidisciplinary Team Requirements

Care must be coordinated by a specialist in skin failure with a multidisciplinary team including dermatology, intensive care physicians, burn surgeons, ophthalmologists, and specialist skincare nurses. 1, 2, 3, 5

• Additional consultations based on organ involvement: otolaryngology, urology/gynecology, wound care, infectious disease. 5

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. 1, 2, 3
• Document 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 national pharmacovigilance authorities. 2
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence. 2
• Schedule ophthalmology follow-up to monitor for chronic ocular complications. 3
• Consider referral to support groups such as SJS Awareness U.K. 2

Common Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality—transfer within hours, not days. 3, 4
• Continuing the culprit medication will worsen the condition and increase mortality. 3
• Overaggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema. 1, 2
• Failure to involve ophthalmology early leads to permanent visual sequelae in over half of patients. 3
• Prophylactic antibiotics increase resistant organism colonization without benefit. 1, 2, 3
• Aggressive debridement of detached epidermis removes the natural biological dressing. 2, 3