What is the treatment for Stevens-Johnson syndrome?

Treatment of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit medications and transfer the patient to a specialized burn unit or ICU, particularly when body surface area involvement exceeds 10%, while initiating comprehensive supportive care as the cornerstone of management. 1, 2

Initial Assessment and Stabilization

Immediate Actions

• Stop all potential causative drugs without delay – this is the single most critical intervention that directly impacts mortality 2, 3
• Calculate SCORTEN on admission to predict mortality risk and guide intensity of care 2, 3
• Transfer patients with >10% body surface area epidermal detachment to a burn center or ICU with experience managing SJS/TEN 1, 2
• Obtain skin biopsy to confirm diagnosis, looking for confluent epidermal necrosis with subepidermal vesicle formation 3

Supportive Environment

• Barrier nursing in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 3
• Monitor vital signs, urine output, and electrolytes regularly 3

Wound and Skin Management

Skin Care Protocol

• Handle skin with extreme gentleness to minimize shearing forces that cause further epidermal detachment 1, 2
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 1, 3
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis, including denuded areas; consider aerosolized formulations to minimize trauma 1
• Leave detached epidermis in situ to act as a biological dressing 1, 3
• Decompress blisters by piercing and expression or aspiration of fluid 1, 3
• Apply nonadherent dressings (Mepitel or Telfa) to denuded dermis 1
• Use secondary foam or burn dressings (Exu-Dry) to collect exudate 1

Infection Management

• Monitor for signs of infection rather than using prophylactic antibiotics, which increase colonization with resistant organisms 2, 3
• Obtain regular skin swabs for culture to detect predominant organisms 3
• Apply topical antimicrobial agents to sloughy areas only, with choice guided by local microbiological advice 1
• Consider silver-containing products/dressings for limited areas (avoid extensive use due to absorption risk) 1

Fluid and Nutritional Support

Fluid Resuscitation

• Provide careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding overaggressive replacement that leads to pulmonary, cutaneous, and intestinal edema 1, 2
• Fluid requirements are lower than predicted by Parkland formula for burns 1

Nutrition

• Deliver continuous enteral nutrition throughout the acute phase, either orally or via nasogastric feeding if buccal mucositis precludes oral intake 1, 3
• Provide 20-25 kcal/kg daily during the early catabolic phase 1, 3
• Increase to 25-30 kcal/kg daily during the anabolic recovery phase 1, 3

Mucosal Care

Ocular Management

• Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews during acute illness 1, 2, 3
• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 1, 2
• Perform daily ocular hygiene by an ophthalmologist or trained nurse to remove inflammatory debris and break down conjunctival adhesions 1, 2
• Administer broad-spectrum topical antibiotics when corneal fluorescein staining or ulceration is present 1, 2
• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 3

Oral Care

• Daily oral review during the acute illness 1
• Apply white soft paraffin ointment to the lips every 2 hours 1, 2
• Clean the mouth daily with warm saline mouthwashes or an oral sponge 1, 2
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 1, 2
• Use antiseptic oral rinse containing chlorhexidine twice daily 1, 2
• Use potent topical corticosteroid mouthwash (betamethasone sodium phosphate) four times daily 1

Urogenital Care

• Daily urogenital review during the acute illness 1, 4
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1, 2, 4
• Use potent topical corticosteroid ointment once daily to involved, noneroded surfaces 1, 2
• Apply silicone dressings (Mepitel) to eroded areas 1, 2
• Consider urinary catheterization when urogenital involvement causes dysuria or retention 3, 4

Systemic Immunomodulatory Therapy

Treatment Options

• Cyclosporine (3 mg/kg daily for 10 days, tapered over 1 month) has shown benefit with reduced mortality compared to predicted rates 2, 3, 5
• Systemic corticosteroids, particularly early IV methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset 2, 3, 5
• For immune checkpoint inhibitor-induced SJS/TEN: administer IV methylprednisolone 0.5-1 mg/kg for grade 3 or 1-2 mg/kg for grade 4, converting to oral corticosteroids on response with taper over at least 4 weeks 1
• IVIG or additional immunosuppression may be considered in severe or steroid-unresponsive cases 1

Airway Management

• Respiratory symptoms and hypoxemia on admission require early intensivist consultation and rapid transfer to ICU or burn center 1, 2
• Perform fiberoptic bronchoscopy to assess airway involvement 1, 2

Discharge Planning and Follow-up

Patient Education and Documentation

• Provide written information about drug(s) to avoid and any potentially cross-reactive medications 1, 2, 3
• Encourage the patient to wear a MedicAlert bracelet bearing the name of the culprit drug 1, 2, 3
• Document drug allergy in the patient's notes and inform all doctors involved in care 1, 2, 3
• Report the episode to national pharmacovigilance authorities 1, 2

Follow-up Appointments

• Organize dermatology outpatient appointment within a few weeks of discharge 1, 2
• Arrange ophthalmology outpatient appointment if ocular involvement occurred 1, 2
• Inform patients about potential fatigue and lethargy for several weeks following discharge 3

Critical Pitfalls to Avoid

• Delayed recognition and discontinuation of the culprit medication significantly increases mortality risk 2, 3
• Indiscriminate use of prophylactic antibiotics increases skin colonization with resistant organisms 2, 3
• Overaggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema 1, 2, 3
• Failure to involve ophthalmology early can lead to permanent ocular sequelae 2, 3
• Continued use of the culprit medication will worsen the condition and increase mortality 3