Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Discontinue the suspected culprit and all nonessential systemic drugs at presentation. Medication-triggered SJS/TEN commonly follows a first exposure by 4 to 28 days; high-risk classes include anti-infective sulfonamides, antiseizure drugs, oxicam NSAIDs, allopurinol, and nevirapine. Record the drug name, start date, dose changes, and last dose before additional medications obscure causality assessment. Nature+1NatureMultiomic single-cell sequencing defines tissue-specific responses in Stevens-Johnson syndrome and toxic epidermal necrolysis | Nature CommunicationsPubMedCurrent Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis - PubMed

Admit patients with progressive epidermal detachment, extensive mucosal involvement, inability to maintain oral intake, respiratory symptoms, hemodynamic instability, or significant skin pain to an inpatient setting capable of intensive monitoring. Transfer to an ICU or burn unit when warranted because core acute management requires fluid replacement, local wound treatment, nutritional support, and respiratory management. Nature+1NatureEffectiveness of early treatment with plasma exchange in patients with Stevens–Johnson syndrome and toxic epidermal necrolysis | Scientific ReportsCDC[PDF] Influenza B virus infection and Stevens–Johnson syndrome

Obtain dermatology consultation and perform a skin biopsy promptly when the diagnosis is uncertain or a competing blistering disorder would alter management. Diagnosis rests on characteristic clinical findings with histology showing full-thickness epidermal necrosis. ccjm+1ccjmToxic epidermal necrolysis and Stevens-Johnson syndrome - PubMedPubMedCurrent Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis - PubMed

Request same-day ophthalmology assessment for any conjunctival, eyelid, visual, or ocular-surface involvement; use lubricating ointment as part of acute ocular care. Ocular, oral, and genital disease is frequent, and long-term complications can include blindness and reproductive morbidity. Nature+2NatureMultiomic single-cell sequencing defines tissue-specific responses in Stevens-Johnson syndrome and toxic epidermal necrolysis | Nature CommunicationsPubMedRetrospective Study of Patients With SJS/TEN Treated at a Tertiary Burn Unit in Canada: Overview of 17 Years of Treatment - PubMedCDC[PDF] Influenza B virus infection and Stevens–Johnson syndrome

Use the combination of prodromal systemic illness, painful skin and mucosal lesions, epidermal blistering or detachment, and a compatible medication exposure to prioritize SJS/TEN. The typical syndrome includes an influenza-like prodrome followed by painful cutaneous and ocular, oral, or genital mucous membrane lesions. PubMedPubMedCurrent Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis - PubMed

Biopsy an active lesional edge for routine histopathology when morphology is compatible with SJS/TEN but diagnostic uncertainty persists. Full-thickness epidermal necrosis supports epidermal necrolysis and helps distinguish this process from conditions in which management may require a materially different approach. ccjm+1ccjmToxic epidermal necrolysis and Stevens-Johnson syndrome - PubMedPubMedCurrent Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis - PubMed

Estimate severity using SCORTEN after identifying SJS/TEN. SCORTEN is available for prognostic severity assessment, but it does not replace repeated bedside reassessment of detachment progression, airway status, fluid balance, nutritional intake, or evolving sepsis. PubMed+1PubMedCurrent Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis - PubMedCDC[PDF] Influenza B virus infection and Stevens–Johnson syndrome

Maintain a specific differential when a presumed SJS/TEN diagnosis does not fit the exposure history, mucosal distribution, or biopsy. Erythema multiforme, autoimmune blistering disease, staphylococcal scalded skin syndrome, and other severe cutaneous adverse reactions require distinct etiologic investigation and may not share the same drug-avoidance plan. ScienceDirect+1ScienceDirectBurn unit care of Stevens Johnson syndrome/toxic epidermal necrolysis: A survey - ScienceDirectCDC[PDF] Influenza B virus infection and Stevens–Johnson syndrome

Construct a medication timeline covering all prescription, over-the-counter, intermittent, and recently discontinued agents. Prioritize drugs started within the preceding 4 to 28 days, but avoid assigning causality solely because a drug is common or temporally adjacent; adverse-drug-reaction causality tools for SJS/TEN have recognized limitations. Nature+1NatureMultiomic single-cell sequencing defines tissue-specific responses in Stevens-Johnson syndrome and toxic epidermal necrolysis | Nature Communicationsaccessdata fda[PDF] Scientific Review for Proposed Administrative Order OTC000035

The leading drug classes include antibiotics, antipyretic analgesics, anticonvulsants, sulfonamides, NSAIDs, allopurinol, and antituberculosis drugs. In Asian populations, carbamazepine, allopurinol, and phenytoin have been especially common culprits; this epidemiology should focus the exposure history but not substitute for individual assessment. Nature+3NatureMultiomic single-cell sequencing defines tissue-specific responses in Stevens-Johnson syndrome and toxic epidermal necrolysis | Nature CommunicationsWileyGuidelines for the Management of Stevens–Johnson Syndrome and ...WileyStevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A ...WileyThe Medication Risk of Stevens–Johnson Syndrome and Toxic ...

Enter the suspected causal agent and reaction phenotype as SJS/TEN in the allergy record before discharge, and provide the patient with written avoidance documentation. Re-exposure to the implicated drug is not appropriate after suspected or confirmed SJS/TEN; future prescribing should use an unrelated alternative when one is available. PubMed+1PubMedCurrent Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis - PubMedPubMedThe Effects of Systemic Cyclosporine in Acute Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis on Ocular Disease - PMC

Use pharmacogenetic prevention prospectively in relevant new-drug starts rather than as an acute diagnostic test. Clinically implemented markers include HLA-B15:02 and HLA-A31:01 for new carbamazepine users, HLA-B58:01 for allopurinol, and HLA-B57:01 for abacavir. PubMedPubMedPharmacogenetic Testing for Prevention of Severe Cutaneous Adverse Drug Reactions

Manage denuded skin and systemic physiologic losses with fluid replacement, local wound treatment, nutritional support, and respiratory management. These interventions constitute the mainstay of treatment and should be delivered in a coordinated ICU, burn-unit, or experienced inpatient environment according to disease severity and local capability. Nature+1NatureEffectiveness of early treatment with plasma exchange in patients with Stevens–Johnson syndrome and toxic epidermal necrolysis | Scientific ReportsCDC[PDF] Influenza B virus infection and Stevens–Johnson syndrome

Screen actively for sepsis rather than treating prophylactically. Systemic prophylactic antibiotics remain controversial; initiate systemic antibiotics when clinical signs and symptoms of sepsis are present, while continuing careful wound surveillance. CDCCDC[PDF] Influenza B virus infection and Stevens–Johnson syndrome

Use multidisciplinary consultation early: dermatology for diagnostic confirmation and skin-directed care, ophthalmology for ocular involvement, and gynecology or urology when genital mucosal disease is present. Published management guidance and clinical series emphasize multidisciplinary involvement, and genital involvement occurred in 60% of one burn-unit cohort. Wiley+1WileyDiagnosis and Management of Stevens‐Johnson ...PubMedRetrospective Study of Patients With SJS/TEN Treated at a Tertiary Burn Unit in Canada: Overview of 17 Years of Treatment - PubMed

Monitor for chronic complications before discharge planning begins. Survivors may develop blindness and respiratory, reproductive, and mental health sequelae; schedule follow-up according to involved epithelial sites rather than considering cutaneous re-epithelialization the endpoint of care. NatureNatureMultiomic single-cell sequencing defines tissue-specific responses in Stevens-Johnson syndrome and toxic epidermal necrolysis | Nature Communications

Do not allow selection of an immunomodulator to delay drug withdrawal, wound care, fluid management, nutrition, airway assessment, or ophthalmology involvement. Corticosteroids, cyclosporine, etanercept, IVIG, and plasma exchange have all been used, but effectiveness remains uncertain and no clear consensus establishes a single preferred systemic regimen. Nature+1NatureEffectiveness of early treatment with plasma exchange in patients with Stevens–Johnson syndrome and toxic epidermal necrolysis | Scientific ReportsPubMedThe Effects of Systemic Cyclosporine in Acute Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis on Ocular Disease - PMC

Cyclosporine has observational and retrospective signals of lower-than-predicted mortality, including a retrospective comparison using 3 to 5 mg/kg/day for up to 7 days. These data are not definitive, and cyclosporine selection should account for renal impairment and the capacity to monitor treatment-related risk. NEJM+2NEJMNew Treatment for Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis? | NEJM ClinicianOxford AcademicRecent progress in Stevens–Johnson syndrome/toxic epidermal ...PubMedThe Effects of Systemic Cyclosporine in Acute Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis on Ocular Disease - PMC

IVIG has been used, including 1 g/kg/day for 3 days in a retrospective treatment comparison, but prospective and comparative reports have not established consistent benefit. A retrospective burn-unit cohort reported higher mortality among patients receiving corticosteroids plus IVIG than among those receiving IVIG alone, but treatment allocation in such studies limits causal interpretation. NEJM+2NEJMNew Treatment for Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis? | NEJM ClinicianNatureHigh-dose intravenous immunoglobulin in the treatment of toxic epidermal necrolysis: a study of ocular benefits | EyePubMedRetrospective Study of Patients With SJS/TEN Treated at a Tertiary Burn Unit in Canada: Overview of 17 Years of Treatment - PubMed

Etanercept and corticosteroid-containing regimens remain options used by some centers, but evidence includes observational experience rather than a settled standard. Plasma exchange has likewise been evaluated; recent comparative observations did not establish benefit over IVIG in corticosteroid-nonresponsive patients. Nature+1NatureEffectiveness of early treatment with plasma exchange in patients with Stevens–Johnson syndrome and toxic epidermal necrolysis | Scientific ReportsScienceDirectEvaluation of Combination Therapy With Etanercept and Systemic Corticosteroids for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Multicenter Observational Study - ScienceDirect

Before discharge, reconcile all medication lists and label the implicated medication as causing SJS/TEN rather than a nonspecific rash. Provide the patient and outpatient prescribers a written list of medications to avoid, because the principal preventable recurrence risk is inadvertent re-exposure to a suspected causal drug. PubMed+1PubMedCurrent Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis - PubMedPubMedThe Effects of Systemic Cyclosporine in Acute Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis on Ocular Disease - PMC

Arrange ophthalmology follow-up after acute ocular involvement because chronic ocular complications can include blindness. Align gynecologic, urologic, respiratory, and mental health follow-up with the epithelial sites involved during the acute illness, as reproductive, respiratory, and mental health sequelae are recognized long-term outcomes. Nature+1NatureMultiomic single-cell sequencing defines tissue-specific responses in Stevens-Johnson syndrome and toxic epidermal necrolysis | Nature CommunicationsWileyDiagnosis and Management of Stevens‐Johnson ...

For future high-risk prescribing, use established pharmacogenetic prevention pathways before initiating selected drugs: HLA-B15:02 and HLA-A31:01 for carbamazepine, HLA-B58:01 for allopurinol, and HLA-B57:01 for abacavir. These tests are prevention tools for new treatment decisions, not proof of the culprit in an acute episode. PubMedPubMedPharmacogenetic Testing for Prevention of Severe Cutaneous Adverse Drug Reactions