What is the management of Stevens-Johnson (Stevens-Johnson Syndrome)?

Management of Stevens-Johnson Syndrome

Immediately discontinue all potential culprit drugs and transfer patients to a specialized burn unit or ICU, particularly when body surface area involvement exceeds 10%, while initiating comprehensive supportive care and early systemic immunomodulation with either cyclosporine or corticosteroids within 72 hours of onset. 1, 2, 3

Initial Assessment and Triage

Immediate Actions

• Stop all suspected medications immediately - this is the single most critical intervention that directly impacts survival 1, 2, 3
• Calculate SCORTEN on admission to predict mortality risk and guide intensity of care 1, 2
• Transfer patients with >10% body surface area epidermal detachment to a burn center or ICU with SJS/TEN experience 2, 3, 4
• Obtain skin biopsy to confirm diagnosis and exclude other blistering disorders (look for confluent epidermal necrosis with subepidermal vesicle formation) 1

Critical History Elements

• Document the exact date of rash onset and progression pattern 1
• Record all medications taken in the previous 2 months, including over-the-counter and herbal products, with start dates 1
• Identify prodromal symptoms: fever, malaise, upper respiratory symptoms preceding painful rash on face and chest 1, 5
• Assess for respiratory symptoms (cough, dyspnea, hemoptysis), bowel involvement (diarrhea, distension), and mucosal pain (eyes, mouth, genitalia) 1

Supportive Care - The Foundation of Management

Environmental and Barrier Nursing

• Maintain room temperature at 25-28°C to reduce transcutaneous water loss 3
• Use pressure-relieving mattress with barrier nursing techniques 1, 2
• Handle skin with extreme gentleness to minimize shearing forces that cause further detachment 2, 3

Fluid and Nutritional Management

• Provide careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding overload that causes pulmonary, cutaneous, and intestinal edema 2, 3
• Monitor vital signs, urine output, and electrolytes regularly 1
• Deliver continuous enteral nutrition: 20-25 kcal/kg daily during catabolic phase, 25-30 kcal/kg during recovery 2
• Use nasogastric feeding when oral intake is precluded by severe buccal mucositis 2

Wound Care Protocol

• Leave detached epidermis in situ to act as a biological dressing 2, 3
• Decompress blisters by piercing and expressing fluid without removing the roof 2
• Irrigate wounds gently with warmed sterile water, saline, or chlorhexidine (1:5000) 2
• Apply bland emollients (petrolatum or dimethicone) frequently over entire epidermis including denuded areas 2, 3
• Use nonadherent dressings (e.g., Mepitel) on denuded dermis with secondary foam or burn dressings to collect exudate 2, 3
• Consider silver-containing dressings only for sloughy areas 2
• High-strength topical corticosteroids may be applied to affected skin 2

Pain Management

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

Infection Prevention and Treatment

Monitor for clinical 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 2
• Use targeted antimicrobial therapy only when clinical infection is present (increased erythema, purulent discharge, fever, positive cultures) 2, 3
• Avoid indiscriminate prophylactic antibiotics - this is a critical pitfall 2, 3

Mucosal Management - Preventing Long-Term Sequelae

Ocular Care (Critical for Preventing Blindness)

• Ophthalmology consultation within 24 hours of diagnosis with daily examinations throughout acute phase 1, 2, 3
• Apply preservative-free lubricant eye drops every 2 hours 2, 3
• Perform daily ocular hygiene by ophthalmologist or trained nurse to remove inflammatory debris and lyse conjunctival adhesions 2, 3
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 2, 3
• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 2

Oral Care

• Perform daily oral reviews during acute illness 3
• Apply white soft paraffin ointment to lips every 2 hours 3
• Clean mouth daily with warm saline mouthwashes or oral sponge 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 (viscous lidocaine 2% or cocaine mouthwashes 2-5%) for severe oral discomfort 2
• Monitor for secondary infections; treat with appropriate antifungals or antivirals if HSV suspected 2

Urogenital Care

• Perform daily urogenital reviews during acute illness 3
• 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 3
• Apply silicone dressings (e.g., Mepitel) to eroded areas 3
• Consider urinary catheterization when dysuria or retention occurs, or to monitor output 2
• Use vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 2

Systemic Immunomodulatory Therapy

First-Line Options (Choose One)

Cyclosporine is recommended based on multiple studies showing reduced mortality compared to predicted rates. 2, 3

• Dose: 3-5 mg/kg daily for 10-14 days, then taper over 1 month 2, 6
• May be used alone or in combination with corticosteroids 6

Systemic corticosteroids may be beneficial if started early (within 72 hours of onset). 2, 3, 6

• Dose: Prednisolone 1-2 mg/kg/day or IV methylprednisolone equivalent 6
• Taper rapidly within 7-10 days 6
• Evidence is stronger when initiated within 72 hours 2, 3

Evidence Considerations

• The British Journal of Dermatology guidelines note that cyclosporin has shown benefit in multiple studies 2
• Indian guidelines recommend moderate to high-dose corticosteroids as first-line 6
• Both approaches have supporting evidence; choice depends on institutional experience and patient factors 7, 6

Respiratory Management

• Assess for respiratory symptoms and hypoxemia on admission 3
• Early discussion with intensivist and rapid ICU transfer if respiratory involvement present 3
• Perform fiberoptic bronchoscopy to assess airway involvement 3

Multidisciplinary Team Approach

Assemble a team including dermatology, intensive care, ophthalmology, and specialist skincare nursing from the outset. 2, 3, 4

• Add additional specialists based on organ involvement (pulmonology, gastroenterology, urology, gynecology) 2

Discharge Planning and Long-Term Follow-Up

Patient Education and Safety

• Provide written information about culprit drug(s) to avoid and potentially cross-reactive medications 2, 3
• Encourage MedicAlert bracelet/amulet bearing the name of the culprit drug 1, 2, 3
• Document drug allergy in medical records and inform all healthcare providers 2, 3
• Report adverse drug reaction to national pharmacovigilance authorities 2, 3

Follow-Up Arrangements

• Schedule dermatology outpatient appointment within a few weeks of discharge 3
• Arrange ophthalmology follow-up if ocular involvement occurred 3
• Inform patients about potential fatigue and lethargy for several weeks requiring convalescence 2
• Consider referral to support groups (e.g., SJS Awareness U.K.) 2

Critical Pitfalls to Avoid

• Delayed recognition and drug discontinuation - significantly increases mortality 2, 3
• Prophylactic antibiotics - increase resistant organism colonization without benefit 2, 3
• Overaggressive fluid resuscitation - causes pulmonary, cutaneous, and intestinal edema 2, 3
• Delayed ophthalmology involvement - leads to permanent visual sequelae including blindness 2, 3
• Continued use of culprit medication - worsens condition and increases mortality 2
• Failure to assess SCORTEN - prevents appropriate risk stratification 1

Quality Metrics for Audit

• SCORTEN performed on admission 1
• Drug causality assessment within 24 hours 1
• Ophthalmology consultation within 24 hours with daily assessments 1
• Daily oral and urogenital assessments during acute phase 1
• GP notification at discharge with drug avoidance counseling and MedicAlert request 1