What is the management and treatment for Stevens-Johnson Syndrome (SJS)?

Management of Stevens-Johnson Syndrome (SJS)

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, 3

Initial Assessment and Triage

• Calculate SCORTEN within the first 24 hours of admission to predict mortality risk and guide intensity of care 1, 2, 3
• 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 with start dates 3
• Obtain skin biopsy from lesional skin adjacent to a blister for histopathology showing confluent epidermal necrosis with subepidermal vesicle formation 1, 3
• Early transfer to specialized centers reduces mortality; delays in specialized care adversely affect outcomes 3

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
• Monitor vital signs, urine output, and electrolytes regularly 1
• Provide continuous enteral nutrition throughout the acute phase: 20-25 kcal/kg daily during the catabolic phase and 25-30 kcal/kg during recovery 1

Fluid Management

• Establish adequate intravenous fluid replacement guided by urine output to prevent end-organ hypoperfusion 1, 2, 3
• Avoid overaggressive fluid resuscitation which causes pulmonary, cutaneous, and intestinal edema 1, 2, 3
• Consider using the formula: body weight/% BSA epidermal detachment to determine replacement volumes 3

Wound Care

• Handle skin with extreme care to minimize shearing forces and prevent further epidermal detachment 1, 2, 3
• Leave detached epidermis in situ to act as a biological dressing 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 every 4 hours 1, 3
• Apply nonadherent dressings (such as Mepitel or Telfa) to denuded dermis with secondary foam or burn dressings to collect exudate 1, 2, 3
• Consider silver-containing products/dressings for sloughy areas only 1

Pain Management

• Provide adequate background simple analgesia to ensure comfort at rest using the WHO analgesic ladder 4
• Add opiates as required, delivered enterally, by patient-controlled analgesia (PCA), or via infusion for moderate-to-severe uncontrolled pain 4
• Consider sedation or general anesthesia for patient handling, repositioning, and dressing changes 3
• Monitor level of consciousness, respiratory rate, and oxygen saturation carefully when using opiate infusions 4

Infection Prevention and Management

Do not administer prophylactic systemic antibiotics as this increases skin colonization with resistant organisms, particularly Candida albicans 1, 2, 3

• Monitor for clinical signs of infection (confusion, hypotension, reduced urine output, reduced oxygen saturation) rather than using prophylactic antibiotics 1, 3
• Obtain regular skin swabs for bacterial and candidal culture from lesional skin to detect predominant organisms 1, 3
• Institute targeted antimicrobial therapy only when clinical signs of infection are present 1, 2, 3
• Watch for monoculture of organisms on culture swabs from multiple sites, indicating increased likelihood of invasive infection 3

Mucosal Management

Ocular Care (Critical Priority)

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
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 1
• 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 1
• Consider amniotic membrane transplantation (AMT) in the acute phase, which demonstrates significantly better visual outcomes compared to medical management alone 1
• Failure to involve ophthalmology early leads to permanent visual impairment 2, 3

Oral Care

• Perform daily oral review during the acute illness 1, 2
• Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness 1, 2
• Clean the mouth daily with warm saline mouthwashes or an oral sponge 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 to reduce bacterial colonization 1, 2
• Apply topical anesthetics such as viscous lidocaine 2% or cocaine mouthwashes 2-5% for severe oral discomfort 1
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 1

Urogenital Care

• Perform daily urogenital review during the acute illness 2
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1, 2
• Use potent topical corticosteroid ointment once daily to involved, noneroded surfaces 2
• Apply silicone dressings (e.g., Mepitel) to eroded areas 2, 3
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1
• Use urinary catheterization when urogenital involvement causes dysuria or retention, or to monitor output 1

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 1, 2

• Systemic corticosteroids, particularly early IV methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset 1, 2
• The evidence for immunomodulating agents remains limited, with high-quality randomized controlled trials still lacking 5, 6
• TNF-α inhibitors show increasing evidence of decreased mortality but require further multicenter trials 6

Additional Supportive Medications

• Provide gastric protection with a proton pump inhibitor in patients where enteral nutrition cannot be established 4
• Administer prophylactic anticoagulation with low molecular weight heparin for immobile patients unless contraindicated 4
• Consider recombinant human G-CSF for neutropenic patients to resist infectious complications and potentially enhance re-epithelialization 4

Airway and Respiratory Management

• Respiratory symptoms and hypoxemia on admission should prompt early discussion with an intensivist and rapid transfer to an ICU or burn center 2
• Perform fibreoptic bronchoscopy to assess airway involvement 2

Multidisciplinary Team Approach

• Coordinate care through a multidisciplinary team led by a specialist in skin failure (dermatology/plastic surgery) 3
• Include clinicians from intensive care, ophthalmology, and specialist skincare nursing as core team members 1, 3
• Add respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, and pharmacy as needed based on organ involvement 3

Discharge Planning and Follow-up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 1, 2
• Encourage patients to wear a MedicAlert bracelet or amulet bearing the name of the culprit drug 1, 2
• Document the drug allergy in the patient's medical records and inform all healthcare providers involved in their care 1, 2
• Report the adverse drug reaction to national pharmacovigilance authorities 1, 2
• Organize dermatology outpatient clinic appointment within a few weeks of discharge 2, 3
• Arrange ophthalmology outpatient appointment if ocular involvement occurred 2, 3
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 1
• Consider referral to support groups such as SJS Awareness U.K. 1

Common Pitfalls to Avoid

• Delayed recognition and discontinuation of the culprit medication significantly increases mortality risk 2, 7
• Indiscriminate use of prophylactic antibiotics increases skin colonization with resistant organisms 1, 2, 3
• Overaggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema 1, 2, 3
• Failure to involve ophthalmology within 24 hours results in permanent ocular sequelae 1, 2
• Delayed transfer to specialized care adversely affects outcomes and increases mortality 3
• Continued use of the culprit medication worsens the condition and increases mortality 2