How is a patient with Steven Johnson Syndrome treated?

Treatment of Stevens-Johnson Syndrome

Immediate Actions

Immediately discontinue all suspected culprit medications and transfer the patient to a specialized burn unit or intensive care unit with multidisciplinary expertise in SJS/TEN management. 1, 2

• Calculate SCORTEN on admission to predict mortality risk and guide intensity of care 1, 2
• Transfer is particularly critical when body surface area involvement exceeds 10% 1, 2
• Early transfer to specialized centers significantly reduces mortality; delays adversely affect outcomes 1, 2
• Specialized centers should include dermatology, intensive care, burn surgery, and ophthalmology expertise 2

Supportive Care Framework

Environmental and General Care

• Barrier nurse the patient in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 1, 2
• Handle skin carefully to minimize shearing forces and prevent further epidermal detachment 1, 2
• Avoid adhesive materials including blood pressure cuffs, ECG leads, adhesive dressings, and identification wrist tags 1
• Use soft silicone tapes for essential clinical items like cannulas and nasogastric tubes 1

Fluid Management

Establish adequate intravenous fluid replacement guided by urine output and end-organ perfusion markers, while carefully avoiding fluid overload. 1, 2

• Monitor fluid balance carefully and catheterize if clinically indicated 1
• Adjust fluid replacement daily with careful monitoring of sodium levels 1
• In severely affected cases, use continuous invasive hemodynamic monitoring through central or arterial lines 1
• Monitor markers of end-organ function including urine output, serial serum lactate, base deficit, and serum urea/electrolytes 1
• Be cautious of overhydration and resultant hyponatremia 1
• Progressively encourage oral fluid administration as mouth involvement improves 1

Wound and Skin Care

Leave detached epidermis in situ to act as a biological dressing, and apply frequent bland emollients to support barrier function. 2

• Decompress blisters by piercing and expressing or aspirating fluid 2
• Apply nonadherent dressings to denuded dermis with secondary foam or burn dressings to collect exudate 2
• Consider silver-containing products/dressings for sloughy areas only 2
• Gently irrigate wounds with warmed sterile water, saline, or chlorhexidine (1/5000) 2
• Apply greasy emollient over the entire epidermis, including denuded areas 2
• Consider petrolatum emollients or dimethicone as alternatives for skin protection 2
• High-strength topical corticosteroids may be applied to affected skin areas 2

Infection Prevention and Management

Monitor for signs of infection rather than using prophylactic antibiotics, which increase skin colonization with resistant organisms, particularly Candida. 2

• Take swabs for bacterial and candidal culture from lesional skin, particularly sloughy or crusted areas, throughout the acute phase 1
• Take viral swabs from eroded areas if HSV infection is suspected 1
• Institute targeted antimicrobial therapy only when clinical signs of infection appear 2
• Fever from SJS/TEN itself complicates detection of secondary sepsis, requiring careful monitoring 2

Pain Management

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

• Use an appropriate validated pain tool to assess pain at least once daily in conscious patients 1
• Administer adequate analgesia using intravenous opioid infusions in those not tolerating oral medication 1
• Administer patient-controlled analgesia where appropriate, with involvement of the acute pain team 1
• Consider sedation or general anesthesia for pain associated with patient handling, repositioning, and dressing changes 1
• Consider keeping the patient sedated and ventilated in the intensive therapy unit for the duration of the acute phase 1

Nutritional Support

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

Mucosal Management

Ocular Care

Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews during the acute illness to prevent permanent ocular sequelae. 1, 2

• 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 2
• 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 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 2
• Apply topical anesthetics such as viscous lidocaine 2% for severe oral discomfort 2
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 1, 2
• Consider topical corticosteroids (betamethasone sodium phosphate 0.5 mg in 10 mL water as a 3-minute rinse-and-spit preparation) four times daily 1
• Monitor for and treat secondary HSV infection if suspected 2

Urogenital Care

Examine the urogenital tract as part of initial assessment with daily documented review during acute illness. 1, 2

• Apply white soft paraffin ointment to urogenital skin and mucosae immediately and every 4 hours through the acute illness 1, 2
• Catheterize all patients to prevent urethral strictures 1
• 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 1
• Insert a dilator or tampon wrapped in Mepitel into the vagina to prevent formation of synechiae 1
• Consider applying a potent topical corticosteroid ointment once daily to involved, noneroded urogenital surfaces 1

Systemic Immunomodulatory Therapy

Consider ciclosporin (3 mg/kg daily for 10 days, tapered over 1 month) 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
• Initiate comprehensive systemic immunomodulation with either cyclosporine or corticosteroids within 72 hours of onset 2
• Current evidence shows no single drug has been found superior or beneficial for all patients 3

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
• 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

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
• High-risk children (extensive epidermal loss >70%, high initial SCORTEN, likely medication cause, underlying malignancy, or previous stem cell transplantation) need quicker transfer to specialized care 1, 2

Common Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality 2
• Indiscriminate use of prophylactic antibiotics increases skin colonization with resistant organisms 2
• Overaggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema 2
• Failure to involve ophthalmology early leads to permanent ocular sequelae 2
• Continued use of the culprit medication worsens the condition and increases mortality 2
• Using adhesive materials causes further epidermal trauma 1