What is the management of a patient with Stevens-Johnson syndrome?

Management of Stevens-Johnson Syndrome

Patients with Stevens-Johnson syndrome (SJS) with >10% body surface area epidermal loss should be admitted without delay to a burn center or intensive care unit with experience treating SJS/TEN and facilities to manage extensive skin loss wound care. 1, 2

Initial Assessment and Management

1. Identify and discontinue the culprit drug immediately

   • Document all medications (including OTC preparations) taken within 2 months prior to symptom onset 1
   • Common culprits include: allopurinol, carbamazepine, lamotrigine, nevirapine, oxicam NSAIDs, phenobarbital, phenytoin, sulfamethoxazole, and sulfasalazine 1

2. Calculate SCORTEN within first 24 hours of admission 1, 2

   • Predicts mortality risk based on seven clinical parameters
   • Higher scores correlate with increased mortality (score of 3 = 32% mortality risk) 1

3. Transfer to specialized care setting

   • Patients with >10% BSA involvement require immediate transfer to burn center or specialized ICU 1
   • Delayed transfer is associated with increased mortality 1

Supportive Care Measures

1. Environmental control

   • Barrier-nurse in a side room with controlled humidity
   • Maintain ambient temperature between 25-28°C
   • Use pressure-relieving mattress 1, 2

2. Skin management

   • Handle skin carefully to minimize epidermal detachment (use anti-shear handling techniques)
   • Leave detached epidermis in situ as biological dressing
   • Apply non-adherent dressings (Mepitel™ or Telfa™) to denuded dermis
   • Gently cleanse wounds with warmed sterile water, saline, or dilute chlorhexidine
   • Apply bland emollient (50% white soft paraffin with 50% liquid paraffin) to entire epidermis 1, 2

3. Fluid management

   • Establish IV access through non-lesional skin
   • Monitor fluid balance carefully
   • Note: fluid requirements are lower than predicted by burn formulas 2

4. Pain management

   • Administer adequate analgesia following WHO analgesic ladder principles
   • Consider opiate-based regimen for moderate-to-severe pain
   • Patient-controlled analgesia (PCA) may be appropriate
   • Monitor consciousness level, respiratory rate, and oxygen saturation when using opiates 2

Specialized Care for Affected Systems

1. Ocular care 2

   • Daily ophthalmological review during acute illness
   • Apply preservative-free lubricants every 2 hours
   • Perform daily ocular hygiene to remove debris and break adhesions
   • Use topical antibiotics if corneal fluorescein staining or ulceration is present
   • Consider topical corticosteroid drops under ophthalmologist supervision

2. Urogenital care 2

   • Examine urogenital tract during initial assessment
   • Apply white soft paraffin to urogenital skin/mucosae every 4 hours
   • Use silicone dressings on eroded areas to reduce pain and prevent adhesions
   • Consider potent topical corticosteroid ointment on non-eroded surfaces

3. Oral care 2

   • Clean mouth daily with warm saline mouthwashes
   • Use benzydamine hydrochloride rinse every 3 hours, particularly before eating
   • Consider topical anesthetic preparations for severe pain
   • Use antiseptic oral rinse twice daily

Infection Prevention and Management

1. Avoid prophylactic systemic antibiotics - only treat when clinical signs of infection are present 2
2. Monitor vigilantly for sepsis - may be masked by disease-associated fever 2
3. Consider prophylactic anticoagulation with low molecular weight heparin for immobile patients 2
4. Consider recombinant human G-CSF for neutropenic patients 2

Systemic Therapies

The optimal systemic therapy remains controversial:

1. Systemic corticosteroids

   • May be considered in early disease phase to curtail progression
   • Risk of increased infection, which is the most common cause of death 3, 4
   • If used, administer IV methylprednisolone 0.5-2 mg/kg depending on severity 2
   • Withdraw as soon as possible to minimize infection risk 4

2. Intravenous immunoglobulin (IVIG)

   • May be considered in severe or steroid-unresponsive cases
   • High-dose regimens (total dose of 3-4 g/kg) appear more effective 2
   • Mechanism may involve blockade of Fas ligand-mediated apoptosis 4

3. Cyclosporine

   • May be considered in severe cases 2, 5

Long-term Follow-up

1. Provide written information about drugs to avoid
2. Encourage wearing a MedicAlert bracelet
3. Document drug allergy clearly in patient records
4. Report the episode to pharmacovigilance authorities
5. Arrange follow-up with dermatology and ophthalmology within weeks of discharge
6. Monitor for long-term sequelae including respiratory, ocular, cutaneous, and urogenital complications 2

Pitfalls to Avoid

1. Delayed recognition and transfer - associated with increased mortality 1, 6
2. Inappropriate fluid management - requirements differ from typical burn patients 2
3. Prophylactic antibiotics - may lead to resistant organisms 2
4. Prolonged corticosteroid use - increases infection risk 4
5. Inadequate mucosal care - can lead to permanent sequelae including blindness and strictures 4

SJS/TEN is a medical emergency requiring prompt multidisciplinary management to reduce mortality and long-term morbidity. Early recognition, immediate withdrawal of culprit drugs, and transfer to specialized care are crucial for improving outcomes.