What is the treatment for Steven 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 in SJS/TEN management, particularly when body surface area involvement exceeds 10%. 1, 2, 3

Initial Assessment and Triage

• Calculate SCORTEN within 24 hours of admission to predict mortality risk and guide intensity of care 1, 2, 4
• Document the exact date of rash onset, progression pattern, and all medications taken in the previous 2 months including over-the-counter and herbal products 4
• Obtain skin biopsy from lesional skin adjacent to a blister for histopathology showing confluent epidermal necrosis with subepidermal vesicle formation 4
• Transfer patients with >10% BSA epidermal detachment to a specialized burn unit or ICU without delay, as early transfer reduces mortality 2, 3, 4

Supportive Care Framework

Environmental and General Care

• Barrier nurse the patient in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 1, 2, 4
• Monitor vital signs, urine output, and electrolytes regularly 1, 2

Fluid Management

• Establish adequate intravenous fluid resuscitation guided by urine output, using the formula: body weight/% BSA epidermal detachment to determine replacement volumes 1, 4
• Avoid overaggressive fluid resuscitation which causes pulmonary, cutaneous, and intestinal edema 1, 2, 4

Wound Care

• Minimize shearing forces when handling skin to prevent further epidermal detachment 1, 2, 3
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 1, 4
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis including denuded areas, considering aerosolized formulations 1, 4
• Leave detached epidermis in situ to act as a biological dressing; decompress blisters by piercing and expression or aspiration of fluid 1, 2
• Apply nonadherent dressings (Mepitel™ or Telfa™) to denuded dermis with secondary foam or burn dressings to collect exudate 1, 3, 4

Infection Prevention and Management

• Do NOT use prophylactic systemic antibiotics as they increase skin colonization with resistant organisms, particularly Candida albicans 1, 2, 4
• Take regular skin swabs for bacterial and candidal culture from lesional skin, particularly sloughy areas 4
• Institute targeted antimicrobial therapy only when clinical signs of infection appear (confusion, hypotension, reduced urine output, reduced oxygen saturation) 1, 2, 4
• Apply topical antimicrobial agents (silver-containing products/dressings) to sloughy areas only, limiting use if extensive areas are being treated due to absorption risk 1

Nutrition

• Provide continuous enteral nutrition throughout the acute phase, either orally or via nasogastric feeding if buccal mucositis precludes oral intake 1, 2
• Deliver 20-25 kcal/kg daily during the early catabolic phase, increasing to 25-30 kcal/kg daily during the anabolic recovery phase 1, 2

Pain Management

• Provide adequate background simple analgesia to ensure comfort at rest 1, 4
• Add opiates as required, delivered enterally, by patient-controlled analgesia (PCA), or via infusion for moderate-to-severe uncontrolled pain 1
• Consider sedation or general anesthesia for patient handling, repositioning, and dressing changes 4

Mucosal Management

Ocular Care (Critical to Prevent Permanent Visual Impairment)

• 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 2, 3
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 2
• Administer 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
• Consider amniotic membrane transplantation (AMT) in the acute phase for significantly better visual outcomes 2

Oral Care

• Examine the mouth as part of initial assessment with daily oral review during acute illness 2
• Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness 2
• 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 2, 3
• Apply topical anesthetics such as viscous lidocaine 2% or cocaine mouthwashes 2-5% for severe oral discomfort 2
• 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 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, 4
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 2

Systemic Immunomodulatory Therapy

Ciclosporin (3 mg/kg daily for 10 days, tapered over 1 month) is recommended as it has shown benefit in multiple studies with reduced mortality compared to predicted rates. 2, 3

• Systemic corticosteroids, particularly early intravenous methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset 2, 3
• If corticosteroids were initiated at an outside facility, taper and discontinue them to reduce infection risk 5

Additional Supportive Medications

• Administer low molecular weight heparin as prophylactic anticoagulation against venous thromboembolism in immobile patients 1
• Provide proton pump inhibitor for gastric protection in patients where enteral nutrition cannot be established 1
• Consider recombinant human G-CSF in neutropenic patients to resist infectious complications and potentially enhance re-epithelialization 1

Multidisciplinary Team Approach

• Coordinate care through a multidisciplinary team led by a specialist in skin failure (dermatology/plastic surgery) 4
• Include clinicians from intensive care, ophthalmology, specialist skincare nursing, and additional specialists as needed (respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, pharmacy) 4, 6

Discharge Planning and Follow-up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 2, 3, 4
• Encourage the patient to wear a MedicAlert bracelet bearing the name of the culprit drug 2, 3, 4
• Document the drug allergy in the patient's medical records and inform all healthcare providers involved in their care 2, 3, 4
• Report the adverse drug reaction to national pharmacovigilance authorities 3, 4
• Organize dermatology and ophthalmology outpatient appointments within a few weeks of discharge 3, 4
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 2

Common Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality risk 2, 4
• Indiscriminate use of prophylactic antibiotics increases skin colonization with resistant organisms 1, 2, 4
• Overaggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema 1, 2, 4
• Failure to involve ophthalmology within 24 hours can lead to permanent visual impairment 2, 3
• Continued use of the culprit medication worsens the condition and increases mortality 2, 3
• Neglecting daily mucosal site reviews (oral, ocular, urogenital) results in preventable complications 4