What is the treatment for Stevens Johnson syndrome triggered by ibuprofen (nonsteroidal anti-inflammatory drug)?

Treatment of Stevens-Johnson Syndrome Triggered by Ibuprofen

Immediately discontinue ibuprofen, admit the patient to a burn unit or ICU with dermatology consultation, and initiate IV methylprednisolone 0.5-1 mg/kg for grade 3 disease or 1-2 mg/kg for grade 4 disease, with a prolonged taper over at least 4 weeks. 1

Immediate Management Steps

Drug Discontinuation and Specialist Consultation

• Stop ibuprofen immediately upon suspicion of SJS/TEN, as this is the single most critical intervention to reduce mortality. 1
• Obtain urgent dermatology consultation for all suspected cases. 1
• Consider that ibuprofen may be falsely implicated if started for prodromal fever and mucosal symptoms (protopathic effect), though true ibuprofen-induced SJS/TEN does occur and can develop rapidly, even after a single dose. 1, 2, 3

Admission and Monitoring

• Admit grade 3 patients (10-30% body surface area involvement) to a burn unit with wound care services. 1
• Admit grade 4 patients (>30% BSA or severe systemic involvement) immediately to burn unit or ICU with dermatology and wound care consultation. 1
• Monitor fluid and electrolyte balance, minimize insensible water losses, and implement infection prevention protocols. 1
• Use serial clinical photography to document progression. 1

Systemic Corticosteroid Therapy

Dosing Protocol

• For grade 3 disease: Administer IV methylprednisolone 0.5-1 mg/kg daily, converting to oral corticosteroids upon response. 1
• For grade 4 disease: Initiate IV methylprednisolone 1-2 mg/kg daily, tapering when toxicity resolves. 1
• Taper corticosteroids over a minimum of 4 weeks to prevent relapse, as SJS/TEN involves T-cell immune-directed toxicity requiring prolonged suppression. 1

Important Distinction from Traditional SJS Management

• The usual prohibition of corticosteroids for drug-induced SJS does not apply here—corticosteroids are warranted and should be offered because the underlying mechanism is T-cell immune-directed toxicity. 1
• Adequate immune suppression is necessary and may be prolonged. 1

Supportive Skin Care

Topical Management

• Apply topical emollients and petrolatum-based products to affected skin. 1
• Use high-strength topical corticosteroids to involved areas. 1
• Consider dimethicone as an alternative to petrolatum. 1
• Administer oral antihistamines for symptomatic relief. 1

Alternative Immunosuppressive Therapy

Second-Line Options for Severe or Refractory Cases

• Consider IVIG or cyclosporine for severe cases or those unresponsive to corticosteroids. 1
• IVIG dosing: 1-2 g/kg total dosage. 1, 4
• Cyclosporine may be used as an alternative immunosuppressive agent. 1, 4

Mucosal and Organ-Specific Management

Ocular Involvement

• Obtain ophthalmology consultation immediately for any ocular involvement to prevent scarring and vision loss. 1
• Remove corneal debris and pseudomembranes gently with moistened cotton-tipped applicator. 1
• Apply topical antibiotic prophylaxis (e.g., moxifloxacin or levofloxacin four times daily). 1
• Consider topical corticosteroid drops (dexamethasone 0.1%) under ophthalmologist supervision. 1
• Establish moisture chamber with polyethylene film for unconscious patients to prevent corneal exposure. 1

Oral Mucosal Care

• Use topical corticosteroid rinses (betamethasone sodium phosphate 0.5 mg in 10 mL water as 3-minute rinse-and-spit, four times daily). 1
• Apply clobetasol propionate 0.05% mixed with Orabase directly to affected areas. 1

Urogenital Involvement

• Obtain early vulval specialist assessment in women to consider dilators for preventing vaginal synechiae. 1
• Apply white soft paraffin ointment every 4 hours to urogenital skin and mucosae. 1
• Use Mepitel dressings to eroded areas; insert dilator or tampon wrapped in Mepitel into vagina to prevent synechiae formation. 1
• Catheterize all patients to prevent urethral strictures. 1
• Apply potent topical corticosteroid ointment once daily to involved, non-eroded urogenital surfaces. 1

Additional Specialist Consultations

• Otolaryngology for nasopharyngeal involvement. 1
• Urology for urethral complications. 1
• Gynecology for vaginal involvement. 1
• Pain or palliative care consultation for severe cases. 1

Future Drug Management

Avoidance Recommendations

• Permanently discontinue ibuprofen and avoid all propionic acid NSAIDs (naproxen, ketoprofen, flurbiprofen, fenoprofen, oxaprozin) due to cross-reactivity within the same chemical class. 1
• Document the reaction clearly in the medical record as severe cutaneous adverse drug reaction. 1
• Avoid rechallenge within the propionic acid class due to potential recurrence that cannot be predicted with current testing. 1

Alternative Analgesic Options

• Consider NSAIDs from different structural classes after appropriate interval and testing. 1
• Note that oxicam NSAIDs (meloxicam, piroxicam) and selective COX-2 inhibitors are most commonly associated with SJS/TEN and should be avoided. 1
• Acetic acid NSAIDs (diclofenac) are also highly associated with severe cutaneous reactions. 1

Diagnostic Testing for Future Drug Use

• Do not perform patch testing or delayed intradermal testing until at least 6 months after complete resolution and at least 4 weeks after discontinuing systemic steroids (>10 mg prednisone equivalent). 5
• Drug challenge with ibuprofen is contraindicated except in extreme circumstances. 5
• Consider referral to specialist drug allergy center for diagnostic testing if avoidance is medically compromising. 1

Critical Pitfalls to Avoid

• Do not delay corticosteroid initiation—the T-cell mediated mechanism requires prompt and adequate immune suppression. 1
• Do not use inadequate corticosteroid duration; minimum 4-week taper is essential to prevent relapse. 1
• Do not assume ibuprofen is innocent if started for prodromal symptoms—while protopathic effect occurs, true ibuprofen-induced SJS/TEN is well-documented and can occur after even a single dose. 1, 2, 3
• Do not prescribe other propionic acid NSAIDs assuming they are safe alternatives—cross-reactivity is well-described. 1

Prognosis Considerations

• Ibuprofen-associated SJS has been reported to have lower fatality rates (6.87%) compared to other NSAIDs but highest hospitalization rates (79.27%). 6
• Early recognition and immediate drug withdrawal decrease risk of death. 1
• Monitor for long-term sequelae including ocular complications, cutaneous scarring, and mucosal strictures. 1, 4