What is the treatment for Steven's Johnson syndrome?

Treatment of Stevens-Johnson Syndrome

Immediately discontinue all suspected causative drugs and transfer patients to a specialized burn unit or ICU, particularly when body surface area involvement exceeds 10%, while initiating meticulous supportive care as the cornerstone of management. 1, 2, 3

Initial Assessment and Triage

• Calculate SCORTEN on admission to predict mortality risk and guide intensity of care—this scoring system directly correlates with expected mortality and should drive resource allocation 1, 2, 3

• Transfer urgently to a specialized center (burn unit or ICU) for patients with >10% body surface area epidermal detachment, as mortality rates drop significantly with specialized care (10% vs 25-70% in general settings) 1, 2, 4

• Barrier nurse in a temperature-controlled room (25-28°C) on a pressure-relieving mattress to minimize heat loss through denuded skin and prevent pressure injuries 2, 3

• Obtain skin biopsy to confirm diagnosis, looking for confluent epidermal necrosis with subepidermal vesicle formation 2

Supportive Care: The Foundation of Treatment

Fluid Management

• Use conservative fluid resuscitation compared to burn formulas—SJS/TEN requires lower volumes than predicted by Parkland formula to prevent pulmonary, cutaneous, and intestinal edema 1

• Monitor vital signs, urine output (target >0.5 mL/kg/hr), and electrolytes regularly to guide fluid administration 1, 2

Wound Management

• Leave detached epidermis in situ to act as a biological dressing—this protects the underlying dermis and accelerates re-epithelialization 1, 2, 3

• Apply greasy emollients (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis including denuded areas, preferably using aerosolized formulations to minimize shearing forces 1, 2, 3

• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 1, 2

• Apply nonadherent dressings (such as silver-releasing antimicrobial dressings changed every 3 days) to denuded dermis with secondary foam or burn dressings to collect exudate 1, 2, 4

• Consider urgent operative debridement with porcine xenograft application if conservative management fails, characterized by clinical deterioration, extension of detachment, or delayed healing—this approach achieved complete re-epithelialization within 18 days in specialized series 1, 5

Infection Prevention and Management

• Do NOT use prophylactic antibiotics—indiscriminate administration increases skin colonization, particularly with Candida albicans and resistant organisms 1, 2, 3, 4

• Monitor carefully for clinical signs of infection: increased skin pain, confusion, hypotension, reduced urine output, reduced oxygen saturation, rising C-reactive protein, or neutrophilia 1

• Obtain regular skin swabs for culture; a monoculture from multiple sites that previously showed mixed growth indicates one organism is becoming predominant and increases likelihood of invasive infection 1

• Use targeted antimicrobial therapy only when clinical signs of infection are present 1, 2, 3

Nutritional Support

• Provide continuous enteral nutrition: 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, 4

Mucosal Management: Critical for Long-term Outcomes

Ocular Care (Most Important for Preventing Permanent Sequelae)

• Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews throughout the acute illness—failure to do this early leads to permanent vision-threatening complications 1, 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—this prevents symblepharon formation 1, 2, 3

• Use broad-spectrum topical antibiotics when corneal fluorescein staining or ulceration is present 1, 2, 3

• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 1, 2

Oral Care

• Apply white soft paraffin ointment to lips every 2 hours 3

• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 2, 3

• Use antiseptic oral rinse containing chlorhexidine twice daily 2, 3

• Apply topical anesthetics such as viscous lidocaine 2% or cocaine mouthwashes 2-5% for severe oral discomfort 2

• Monitor for and treat secondary infections; consider HSV activation in eroded areas that are slow to heal 1, 2

Urogenital Care

• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 2, 3

• Use potent topical corticosteroid ointment once daily to involved, noneroded surfaces 2, 3

• Apply silicone dressings (e.g., Mepitel) to eroded areas 2, 3

• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 2

• Use urinary catheterization when urogenital involvement causes dysuria or retention, or to monitor output 2

Systemic Immunomodulatory Therapy: Controversial but Consider Early

The evidence for systemic therapy remains limited, with no randomized controlled trials showing clear benefit except one trial showing harm with thalidomide 1. However, based on observational data:

First-Line Systemic Options (Choose One if Starting Within 72 Hours)

• Cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month—multiple studies show reduced mortality compared to predicted SCORTEN rates 2, 3, 6, 7

• Systemic corticosteroids (IV methylprednisolone pulse therapy)—may be beneficial if started within 72 hours of onset, though evidence is mixed 2, 3, 6, 7

• Stop steroids if initiated at an outside facility and taper rapidly—prolonged steroid use may increase infection risk 4

Intravenous Immunoglobulin (IVIg)

• Consider IVIg, though evidence is equivocal—pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 1

• If using IVIg, high-dose regimens (total 2 g/kg over 3-5 days) showed lower mortality than low-dose in observational studies, though multivariate analysis did not confirm dose-dependent benefit 1

• Pediatric patients treated with IVIg had significantly lower mortality than adults (0% vs 21.6%) 1

Respiratory Management

• Respiratory symptoms and hypoxemia on admission require urgent intensivist consultation and rapid ICU transfer—deterioration requiring mechanical ventilation is likely and carries poor prognosis 1, 3

• Perform fiberoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, investigate pneumonitis, and mechanically remove sloughed bronchial epithelium 1

• Monitor patients with ongoing respiratory symptoms closely with pulmonary function testing and high-resolution CT scanning 1

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 national pharmacovigilance authorities 2, 3

• Organize dermatology and ophthalmology outpatient appointments within a few weeks of discharge 3

• 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

Common Pitfalls to Avoid

• Delayed recognition and discontinuation of the culprit medication—every day of continued exposure significantly increases mortality risk 2, 3, 6

• Overaggressive fluid resuscitation—unlike thermal burns, SJS/TEN requires less fluid; excess causes pulmonary, cutaneous, and intestinal edema 1, 2, 3

• Prophylactic antibiotics—this practice increases colonization with resistant organisms and Candida without preventing infection 1, 2, 3, 4

• Delayed ophthalmology consultation—permanent vision-threatening sequelae develop rapidly; consultation must occur within 24 hours 1, 2, 3

• Aggressive handling of skin—minimize shearing forces as any manipulation can extend epidermal detachment 1, 2, 3

• Failure to calculate SCORTEN—this score should guide triage decisions and family counseling about prognosis 1, 2, 3