What is the appropriate management for a patient suspected to have Steven Johnson syndrome?

Management of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit medications and transfer the patient without delay to a specialized burn center or intensive care unit with multidisciplinary expertise in managing extensive skin loss, particularly when body surface area involvement exceeds 10%. 1, 2

Immediate Life-Saving Actions

• Stop the offending drug immediately – this is the single most critical intervention that directly impacts mortality. 1, 2 The FDA specifically warns that carbamazepine, lamotrigine, and other high-risk medications should be discontinued at the first sign of rash and never resumed if SJS/TEN is suspected. 3

• Calculate SCORTEN within 24 hours to predict mortality risk (ranging from 1% with score 0 to 99% with score 7) and guide intensity of care. 1, 2 This scoring system uses seven parameters including age, malignancy, heart rate, body surface area involvement, serum glucose, bicarbonate, and urea levels. 1

• Transfer to specialized care immediately – patients with >10% body surface area (BSA) epidermal detachment require urgent transfer to a burn center or ICU experienced in SJS/TEN management. 1, 2, 4 Delayed transfer significantly increases mortality risk. 2, 4

Specialized Care Environment

Barrier-nurse the patient in a temperature-controlled side room (25-28°C) on a pressure-relieving mattress with humidity control. 1, 2 This prevents hypothermia and reduces infection risk, as septicemia is the leading cause of death. 1, 5

Multidisciplinary Team Coordination

Coordinate care through a team led by a dermatologist or plastic surgeon specializing in skin failure, including: 1, 2

• Intensive care physicians
• Ophthalmologist (consultation within 24 hours is mandatory) 1, 2
• Specialist skincare nurses
• Additional specialists as needed: respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, pharmacy 1, 2

Supportive Care Framework

Fluid Management

• Establish adequate intravenous fluid replacement guided by urine output and hemodynamic parameters, using urinary catheterization when indicated. 2, 4

• Avoid overaggressive fluid resuscitation – this causes pulmonary, cutaneous, and intestinal edema. 1, 2, 4 Careful monitoring is essential as SJS/TEN patients are prone to both hypovolemia and fluid overload. 1

Wound Care Protocol

• Handle skin with extreme care to minimize shearing forces that cause further epidermal detachment. 1, 2, 4 Day-to-day care should be performed by specialist nurses familiar with skin fragility disorders. 1

• Leave detached epidermis in situ to act as a biological dressing. 2, 4 Decompress blisters by piercing and expressing fluid without removing the overlying skin. 2

• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis including denuded areas every few hours. 2, 4 This supports barrier function, reduces transcutaneous water loss, and encourages re-epithelialization. 1, 2

• Gently irrigate wounds with warmed sterile water, saline, or chlorhexidine (1/5000). 2, 4

• Apply nonadherent dressings (such as Mepitel or Telfa) to denuded dermis with secondary foam or burn dressings to collect exudate. 2, 4 This reduces fluid and protein loss, limits microbial colonization, controls pain, and accelerates re-epithelialization. 1, 2

Infection Prevention and Management

Do not use prophylactic systemic antibiotics – this is a critical pitfall that increases skin colonization with resistant organisms, particularly Candida albicans. 1, 2, 4 This recommendation is consistently emphasized across all guidelines.

• Monitor for clinical signs of infection including confusion, hypotension, reduced urine output, reduced oxygen saturation, rising C-reactive protein, and neutrophilia. 2, 4

• Take swabs for bacterial and candidal culture from three lesional areas on alternate days. 2, 4

• Institute targeted antimicrobial therapy only when clinical signs of infection appear. 1, 2, 4 Monoculture of organisms on swabs from multiple sites indicates increased likelihood of invasive infection requiring treatment. 4

Nutrition Support

• Provide continuous enteral nutrition throughout the acute phase: 2
  • 20-25 kcal/kg daily during the catabolic phase 2
  • 25-30 kcal/kg daily during the anabolic recovery phase 2
  • Consider nasogastric feeding when oral intake is precluded by buccal mucositis 2

Pain Management

• Use validated pain assessment tools at least once daily. 2, 4

• Provide adequate background analgesia with simple analgesics to ensure comfort at rest, plus additional opioid analgesia for breakthrough pain. 1, 2

• Consider intravenous opioid infusions for those not tolerating oral medication, patient-controlled analgesia where appropriate, or sedation/general anesthesia for patient handling, repositioning, and dressing changes. 2, 4

Mucosal Management

Ocular Care (Critical for Preventing Permanent Blindness)

Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews throughout the acute illness. 1, 2 Failure to provide early and ongoing eye care results in permanent visual impairment. 2

• 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. 2 This prevents symblepharon formation and permanent scarring. 1

• Use 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, 2

• Consider amniotic membrane transplantation (AMT) in the acute phase, which demonstrates significantly better visual outcomes compared to medical management alone. 2

Oral Care

• 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

• Use antiseptic oral rinse 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. 2

• Perform daily oral review during the acute illness. 2

Urogenital Care

• Perform regular examination of urogenital tract during acute illness. 1, 2

• Use urinary catheterization when urogenital involvement causes dysuria or retention, or to monitor output. 1, 2

• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours. 1

• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation. 1

Systemic Immunomodulatory Therapy Options

The evidence for systemic therapies remains equivocal, but when considering intervention:

• Cyclosporine (3 mg/kg daily for 10 days, tapered over 1 month) has shown benefit in multiple studies with reduced mortality compared to predicted rates. 2 This is the most consistently supported systemic therapy in the guidelines.

• Systemic corticosteroids, particularly early IV methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset. 1, 2 However, the evidence is mixed.

• Intravenous immunoglobulin (IVIg) has equivocal evidence – pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75). 2

• Avoid thalidomide – it was associated with excess deaths in one randomized trial. 2

Airway and Respiratory Management

• Respiratory symptoms and hypoxemia on admission should prompt urgent discussion with an intensivist and rapid transfer to ICU or burn center. 2

• Perform fiberoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, investigate pneumonitis, and mechanically remove sloughed bronchial epithelium. 2

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients. 2

• Provide proton pump inhibitor if enteral nutrition cannot be established. 2

• Consider recombinant human G-CSF for neutropenic patients. 2

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 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. 2

• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence. 2

• Arrange follow-up with dermatology and ophthalmology within weeks of discharge. 2

• Consider referral to support groups such as SJS Awareness U.K. 2

Special Considerations for Pediatric Patients

• Infection causes up to 50% of pediatric SJS/TEN cases – test for infective triggers (particularly Mycoplasma pneumoniae) and consult infectious disease team in all pediatric cases. 2, 6

• Manage pediatric patients in age-appropriate specialist units with pediatric intensivists and skin loss specialists. 2

• Consider targeted antibiotics as appropriate (e.g., azithromycin for mycoplasma). 2

Common Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality. 2, 4

• Continued use of the culprit medication will worsen the condition and increase mortality. 1

• 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 early leads to permanent visual sequelae. 1, 2

• Aggressive handling of fragile skin causes further epidermal detachment. 1, 2

• Failure to recognize sepsis – the most common cause of death in SJS/TEN. 1, 4