What is the best course of treatment for a patient presenting with Stevens-Johnson Syndrome (SJS) starting from the lips?

Management of Stevens-Johnson Syndrome Starting from the Lips

For SJS presenting with lip involvement, immediately discontinue all suspected culprit drugs, apply white soft paraffin ointment to the lips every 2 hours, and transfer the patient to a specialized burn unit or ICU if body surface area involvement exceeds 10%. 1, 2

Immediate Lip and Oral Care

The lips are often the first site of mucosal involvement in SJS and require aggressive early management to prevent fibrotic scarring:

• Apply white soft paraffin ointment to the lips immediately upon diagnosis, then every 2 hours throughout the acute illness to reduce the risk of permanent fibrotic scars 3, 2
• Clean the mouth daily with warm saline mouthwashes or an oral sponge 3
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating, to manage pain and inflammation 3, 2
• Apply antiseptic oral rinse containing chlorhexidine twice daily to reduce bacterial colonization of the mucosa 3, 2

For severe oral pain that prevents eating or drinking:

• Consider topical anesthetics such as viscous lidocaine 2% or cocaine mouthwashes 2-5% 2
• For patients unable to use mouthwash (infants or those with severe involvement), apply clobetasol propionate 0.05% cream or ointment directly to affected lip areas 3
• Use potent topical corticosteroid mouthwash such as betamethasone sodium phosphate four times daily for more severe inflammation 3

Critical Initial Actions

Calculate SCORTEN within the first 24 hours to predict mortality risk and guide intensity of care 1, 2

Immediately discontinue all potential culprit medications - this is the single most important intervention that affects survival 2, 4, 5

Common high-risk drugs include:

• Sulfonamide antibiotics (especially trimethoprim-sulfamethoxazole) 5, 6
• Anti-epileptic drugs (carbamazepine, phenytoin, phenobarbital) 5, 6
• Allopurinol 5, 6
• NSAIDs of the oxicam type 5, 6

Transfer Criteria and Care Setting

Transfer patients with >10% body surface area epidermal detachment to a specialized burn center or ICU within hours of diagnosis - delayed transfer significantly increases mortality 1, 2

The patient must be:

• Barrier-nursed in a temperature-controlled side room (25-28°C) on a pressure-relieving mattress 1, 2
• Managed by a multidisciplinary team including dermatology, intensive care, ophthalmology, and specialist skincare nursing 1, 2

Comprehensive Skin and Wound Management

Handle all skin with extreme care to minimize shearing forces that cause further epidermal detachment 1, 2

Wound Care Protocol:

• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 1
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis including denuded areas every few hours 1, 2
• Leave detached epidermis in situ to act as a biological dressing - do not remove it 1, 2
• Decompress blisters by piercing and expressing or aspirating fluid, but leave the blister roof intact 1
• Apply nonadherent dressings (Mepitel™ or Telfa™) to denuded dermis 1
• Use secondary foam or burn dressings (Exu-Dry™) to collect exudate 1
• Apply topical antimicrobial agents only to sloughy areas - consider silver-containing products/dressings, but limit use if extensive areas are being treated due to absorption risk 1

Infection Prevention - Critical Pitfall to Avoid

Do NOT use prophylactic systemic antibiotics - this is a common error that increases skin colonization with resistant organisms, particularly Candida albicans 1, 2, 7

Instead:

• Take swabs for bacterial and candidal culture from three lesional areas on alternate days throughout the acute phase 1
• Administer systemic antibiotics only when clinical signs of infection appear (rising C-reactive protein, neutrophilia, fever with hemodynamic instability) 1, 2
• Monitor carefully as fever from SJS/TEN itself complicates detection of secondary sepsis 2

Ophthalmology Consultation - Non-Negotiable

Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews throughout the acute illness 1, 2

Ocular management:

• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 2
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 2
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 2
• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 2
• Consider amniotic membrane transplantation in the acute phase for significantly better visual outcomes 2

Failure to involve ophthalmology early leads to permanent sequelae including blindness 2

Fluid and Nutritional Support

Fluid Management:

• Establish adequate IV fluid replacement guided by urine output and hemodynamic parameters 1, 2
• Avoid overaggressive fluid resuscitation - this leads to pulmonary, cutaneous, and intestinal edema 1, 2
• Monitor fluid balance with urinary catheterization when urogenital involvement causes dysuria/retention 1

Nutrition:

• Provide continuous enteral nutrition throughout the acute phase - oral route preferred, but use nasogastric feeding if buccal mucositis precludes oral intake 1, 2
• Deliver 20-25 kcal/kg daily during the early catabolic phase 1, 2
• Increase to 25-30 kcal/kg daily during the anabolic recovery phase 1, 2

Enteral nutrition is preferable to parenteral to reduce peptic ulceration and limit translocation of gut bacteria 1

Pain Management

• Use validated pain assessment tools at least once daily 1, 2
• Provide adequate background analgesia to ensure comfort at rest with additional opioid analgesia for breakthrough pain 1, 2
• Additional analgesia is needed for increased pain associated with patient handling, repositioning, and dressing changes 1

Systemic Immunomodulatory Therapy - Evidence Review

The evidence for systemic therapy remains controversial, but recent data favors early intervention:

Corticosteroids:

• IV methylprednisolone 0.5-1 mg/kg may be beneficial if started within 72 hours of onset 1, 2, 4
• For severe cases (grade 4), use IV methylprednisolone 1-2 mg/kg, tapering when toxicity resolves 1
• The usual prohibition of corticosteroids for SJS does not apply to immune checkpoint inhibitor-induced cases, as the underlying mechanism is T-cell immune-directed toxicity 1

Cyclosporine:

• Cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month, has shown benefit with reduced mortality compared to predicted rates 2, 4

IVIG:

• Evidence is equivocal - pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 2
• May be considered in severe or steroid-unresponsive cases 1

Avoid:

• Thalidomide is contraindicated - associated with excess deaths in randomized trial 2

Additional Supportive Measures

• Administer low molecular weight heparin for immobile patients as prophylactic anticoagulation 1, 2
• Provide proton pump inhibitor if enteral nutrition cannot be established to reduce stress-related gastrointestinal ulceration 1, 2
• Consider recombinant human G-CSF for neutropenic patients 2

Urogenital Care

• Examine urogenital tract regularly during acute illness 2
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 2
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 2

Discharge Planning

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 2
• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug 2
• Document the drug allergy in the patient's medical records and inform all healthcare providers 2
• Report the adverse drug reaction to pharmacovigilance authorities 2
• Inform patients about potential fatigue and lethargy for several weeks following discharge 2

Common Pitfalls Summary

1. Delayed recognition and transfer - significantly increases mortality 2
2. Indiscriminate prophylactic antibiotics - increases resistant organism colonization 1, 2
3. Overaggressive fluid resuscitation - causes pulmonary, cutaneous, and intestinal edema 1, 2
4. Failure to involve ophthalmology within 24 hours - leads to permanent ocular sequelae 2
5. Continued use of culprit medication - worsens condition and increases mortality 2
6. Inadequate lip care - leads to permanent fibrotic scarring 3, 2