What is the treatment for Steven Johnson's syndrome (SJS)?

Treatment of Stevens-Johnson Syndrome

Immediate Actions

Immediately discontinue all suspected culprit drugs and transfer the patient to a specialized burn unit or ICU with experience managing SJS/TEN, particularly when body surface area involvement exceeds 10%. 1, 2

• Calculate SCORTEN on admission to predict mortality risk and guide intensity of care 1, 2
• Transfer without delay to centers with multidisciplinary teams including dermatology, intensive care, burn surgery, and ophthalmology 1
• Early transfer reduces mortality; delays in specialized care adversely affect outcomes 1

Supportive Care Framework

Environmental and Barrier Nursing

• Barrier nurse in temperature-controlled room heated to 25-28°C 1, 2
• Use pressure-relieving mattress to prevent further skin damage 1, 3
• Minimize all shearing forces when handling or moving the patient 1, 2

Fluid 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 2

Wound Care

• Leave detached epidermis in situ to act as a biological dressing 2, 3
• Apply bland emollients frequently over entire epidermis to support barrier function and reduce transcutaneous water loss 1, 2
• Use nonadherent dressings on denuded dermis with secondary foam or burn dressings to collect exudate 2, 3
• Gently irrigate wounds with warmed sterile water, saline, or chlorhexidine (1/5000) 2
• Consider silver-containing dressings only for sloughy areas 2

Infection Management

Do not use prophylactic antibiotics as they increase skin colonization with resistant organisms, particularly Candida. 1, 2

• Obtain regular skin swabs for culture to detect predominant organisms 2
• Institute targeted antimicrobial therapy only when clinical signs of infection appear (confusion, hypotension, reduced urine output, reduced oxygen saturation) 1, 2
• Monitor carefully as fever from SJS/TEN itself complicates detection of secondary sepsis 1

Nutritional Support

• Deliver continuous enteral nutrition throughout acute phase: 20-25 kcal/kg daily during catabolic phase, 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

Ocular Care (Critical Priority)

Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews throughout the acute illness. 2, 3

• Apply preservative-free lubricant eye drops every 2 hours throughout acute illness 2, 3
• Perform daily ocular hygiene by ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 2, 3
• Use broad-spectrum 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, 3
• Failure to involve ophthalmology early leads to permanent sequelae including blindness 2, 3

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
• Clean mouth daily with warm saline mouthwashes or oral sponge 3

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

Systemic Pharmacologic Therapy

Ciclosporin (3 mg/kg daily for 10 days, tapered over 1 month) is the recommended systemic therapy, showing reduced mortality compared to predicted rates in multiple studies. 2, 3, 4

• Systemic corticosteroids, particularly IV methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset 2, 3, 5
• The controversy over corticosteroids remains: they may suppress progression but enhance infection risk 6
• If corticosteroids are used, administer only during progression phase and withdraw as soon as possible 6
• Combination of intravenous immunoglobulin (IVIg) and corticosteroids may be most effective for SJS/TEN overlap and TEN 4

Airway Management

• Respiratory symptoms and hypoxemia on admission require early intensivist discussion and rapid ICU/burn center transfer 3
• Perform fiberoptic bronchoscopy to assess airway involvement 3

Discharge Planning and Follow-up

• Provide written information about culprit drug(s) to avoid and potentially cross-reactive medications 2, 3
• Encourage patient to wear MedicAlert bracelet bearing name of culprit drug 2, 3
• Document drug allergy in patient's medical records and inform all healthcare providers 2, 3
• Report adverse drug reaction to national pharmacovigilance authorities 2, 3
• Organize dermatology outpatient appointment within 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 2

Special Considerations for Pediatric Patients

• Infection causes up to 50% of pediatric SJS/TEN cases (HSV, Mycoplasma pneumoniae) 1
• Test for infective triggers and consult infectious disease team in all pediatric cases 1
• Manage in age-appropriate specialist units with pediatric intensivists and skin loss specialists 1
• High-risk children (>70% epidermal loss, high SCORTEN, underlying malignancy, previous stem cell transplant) need quicker transfer to specialized care 1

Common Pitfalls to Avoid

• Continued use of culprit medication worsens condition and increases mortality 2, 3
• Delayed recognition and transfer to specialized care significantly increases mortality 1, 2
• Indiscriminate prophylactic antibiotics increase resistant organism colonization 1, 2
• Overaggressive fluid resuscitation causes pulmonary, cutaneous, and intestinal edema 2, 3
• Failure to involve ophthalmology within 24 hours leads to permanent ocular sequelae 2, 3