What is the treatment for Steven's Johnson syndrome?

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, particularly when body surface area involvement exceeds 10%, while initiating comprehensive supportive care as the cornerstone of management. 1, 2

Initial Assessment and Triage

• Calculate SCORTEN on admission to predict mortality risk and guide intensity of care 1, 2, 3
• Transfer without delay to centers with multidisciplinary teams including dermatology, intensive care, burn surgery, and ophthalmology—early transfer reduces mortality while delays adversely affect outcomes 2, 4
• Barrier nurse patients in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 1, 2
• Obtain skin biopsy to confirm diagnosis, looking for confluent epidermal necrosis with subepidermal vesicle formation 2

Supportive Care: The Foundation of Treatment

Fluid Management

• Provide careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding overaggressive replacement that leads to pulmonary, cutaneous, and intestinal edema 1, 2, 3
• Fluid requirements are lower than burn formulas predict—avoid using Parkland formula 1
• Monitor vital signs, urine output, and electrolytes regularly 2

Wound Care

• Minimize shearing forces when handling skin to prevent further epidermal detachment 1, 2, 3
• Leave detached epidermis in situ to act as a biological dressing 1, 3
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 1
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis including denuded areas every few hours 1, 2
• Apply nonadherent dressings to denuded dermis with secondary foam or burn dressings to collect exudate 1, 3
• Consider silver-containing dressings for sloughy areas only 1, 4
• Decompress blisters by piercing and expressing fluid 1

Infection Prevention and Management

• Do not use prophylactic antibiotics—indiscriminate administration increases skin colonization with resistant organisms, particularly Candida albicans 1, 2, 4
• Institute targeted antimicrobial therapy only when clinical signs of infection appear (confusion, hypotension, reduced urine output, reduced oxygen saturation, increased skin pain) 1, 2
• Obtain regular skin swabs for culture to detect predominant organisms 2, 5
• Monitor for rising C-reactive protein and neutrophilia as indicators of sepsis 1
• Consider HSV activation in eroded or vesicular areas slow to heal, particularly in genital and oral sites 1

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

Mucosal Management

Ophthalmological Care (Critical for Preventing Permanent Sequelae)

• Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews throughout the acute illness 1, 2, 3
• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 1, 2, 3
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 2, 3
• Use 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
• Consider amniotic membrane transplantation in the acute phase for significantly better visual outcomes 2

Oral Care

• Perform daily oral review during the acute illness 1, 3
• Apply white soft paraffin ointment to the lips every 2 hours 1, 2, 3
• Clean the mouth daily with warm saline mouthwashes or an oral sponge 1, 3
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 1, 2, 3
• Use antiseptic oral rinse containing chlorhexidine twice daily 1, 2, 3
• Use potent topical corticosteroid mouthwash (e.g., betamethasone sodium phosphate) four times daily 1
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 2

Urogenital Care

• Perform daily urogenital review during the acute illness 1, 3
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1, 2, 3
• Use potent topical corticosteroid ointment once daily to involved, noneroded surfaces 1, 3
• Apply silicone dressings (e.g., Mepitel) to eroded areas 1, 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

Corticosteroids

• Systemic corticosteroids, particularly early methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset 2, 3, 6
• The FDA label indicates prednisone is approved for severe erythema multiforme (Stevens-Johnson syndrome) 7
• Indian guidelines recommend moderate to high doses of oral or parenteral corticosteroids (prednisolone 1-2 mg/kg/day or equivalent), tapered rapidly within 7-10 days 6
• If steroids were initiated at an outside facility, consider tapering and discontinuing them 4
• Evidence remains mixed—some centers report success while others avoid steroids 8, 9

Cyclosporine

• Cyclosporine (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, 3
• Alternative dosing: 3-5 mg/kg/day for 10-14 days, either alone or in combination with corticosteroids 6
• Increasing evidence shows cyclosporine decreases mortality 9

Intravenous Immunoglobulin (IVIg)

• IVIg has been used but evidence is equivocal—pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 1
• One burn center reported using IVIg in all patients after 2001 as part of their protocol with 10% mortality 4
• Pediatric patients treated with IVIg had significantly lower mortality than adults (0% vs. 21.6%) 1

TNF-α Inhibitors

• Increasing evidence shows TNF-α inhibitors decrease mortality 9
• Thalidomide (an anti-TNF agent) was associated with excess deaths in one randomized trial and should be avoided 1

Airway Management

• Respiratory symptoms and hypoxemia on admission should prompt urgent discussion with an intensivist and rapid transfer to ICU or burn center—deterioration requiring mechanical ventilation is likely 1, 3
• Perform fibreoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, investigate pneumonitis, and mechanically remove sloughed bronchial epithelium 1
• Counsel relatives about the prognostic significance of respiratory involvement 1

Discharge Planning and Follow-up

• Provide written information about 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 1, 2, 3
• Report the adverse drug reaction to national pharmacovigilance authorities 1, 2, 3
• Organize dermatology outpatient appointment within a few weeks of discharge 1, 3
• Arrange ophthalmology outpatient appointment if ocular involvement occurred 1, 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 transfer to specialized care significantly increases mortality 2, 4
• Continued use of the culprit medication will worsen the condition and increase mortality 2
• Indiscriminate prophylactic antibiotics increase resistant organism colonization, particularly Candida 1, 2
• Overaggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema 1, 2, 3
• Failure to involve ophthalmology within 24 hours can lead to permanent ocular sequelae 2, 3
• Using burn formulas (like Parkland) for fluid resuscitation overestimates requirements 1

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 need quicker transfer to specialized care 2