# Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Suspected SJS/TEN requires immediate withdrawal of plausible culprit drugs, clinical-pathologic confirmation, severity assessment, and early multidisciplinary supportive care. Management priorities are transfer to an experienced intensive or burn-care setting, meticulous mucosal surveillance, and prevention of ocular, infectious, respiratory, and genitourinary sequelae.

**Clinical question:** How should physicians rapidly diagnose, triage, stabilize, and manage suspected Stevens-Johnson syndrome or toxic epidermal necrolysis?

Updated: 2026-09-15T17:34:48.720276+00:00

## What matters in practice
- Stop every nonessential medication with a plausible temporal relationship immediately; SJS/TEN is most often drug induced, and delayed onset after first exposure is commonly 4 to 28 days. [5][18]
- Classify epidermal detachment clinically: SJS involves less than 10% body surface area, overlap 10% to 30%, and TEN more than 30%. [1][18]
- Confirm the diagnosis with dermatology assessment and skin biopsy demonstrating full-thickness epidermal necrosis; use clinical morphology and pathology together rather than either alone. [17][18]
- Treat as a dermatologic critical illness: fluid replacement, wound care, nutritional and respiratory support, and escalation to intensive or burn-unit care when warranted are core management. [4][21]
- Obtain urgent ophthalmology involvement for ocular disease and provide ocular lubrication; ocular involvement is common and may result in blindness or chronic visual morbidity. [5][19][21]
- No systemic immunomodulatory therapy has definitive consensus support; cyclosporine, corticosteroids, etanercept, IVIG, and plasma exchange have variable and largely nondefinitive evidence. [2][4][20]

## What to do in the first hours of suspected SJS/TEN

Do not await biopsy results before removing likely triggers and arranging higher-acuity care.

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. [5][18]

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. [4][21]

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. [17][18]

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. [5][19][21]
- Assess the oral, ocular, genital, and respiratory mucosa at presentation; mucous membrane involvement occurs in more than 90% of SJS/TEN cases. [1]
- Document the percentage of detachable or detached epidermis at baseline and serially because detachment extent determines the SJS/TEN category. [1][18]
- Avoid routine systemic prophylactic antibiotics; initiate systemic antibiotics for clinical evidence of sepsis rather than for denuded skin alone. [21]

*Clinical detachment categories used to classify epidermal necrolysis. [1][18]*

| Category | Epidermal detachment | Immediate implication |
| --- | --- | --- |
| Stevens-Johnson syndrome | <10% body surface area [1][18] | Urgent drug withdrawal, biopsy when needed, mucosal examination, and close progression monitoring. [17][18] |
| SJS/TEN overlap | 10%–30% body surface area [18] | Escalate supportive-care planning because disease burden may progress across the spectrum. [4][18] |
| Toxic epidermal necrolysis | >30% body surface area [1][18] | Manage as a life-threatening dermatologic emergency with ICU or burn-unit-level capability when warranted. [4][21] |

## Confirm epidermal necrolysis and separate it from competing blistering disorders

Painful mucocutaneous disease plus detachment should trigger parallel clinical classification and biopsy.

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. [18]

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. [17][18]

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. [18][21]

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. [16][21]
- Use body surface area detachment—not erythema alone—to assign SJS, overlap, or TEN category. [1][18]
- Use histology to support rather than delay clinical action; culprit-drug withdrawal and critical supportive measures begin on suspicion. [4][17][20]
- Reassess oral, ocular, genital, and respiratory involvement serially because multiple epithelial surfaces may be involved and chronic complications are substantial. [5]

*Findings that change the diagnostic next step in suspected epidermal necrolysis. [17][18][21]*

| Finding | Interpretation | Next action |
| --- | --- | --- |
| Painful eruption with ocular, oral, or genital mucosal disease after a new systemic medication | Raises concern for drug-induced SJS/TEN. [5][18] | Stop plausible culprit drugs, assess detachment extent, and obtain dermatology evaluation. [4][18] |
| Clinical detachment with uncertain morphology or atypical exposure history | SJS/TEN remains possible, but competing blistering disorders require exclusion. [16][17] | Perform skin biopsy for histopathology and direct management to the confirmed process. [17] |
| Full-thickness epidermal necrosis on biopsy | Supports epidermal necrolysis when aligned with clinical findings. [17][18] | Classify by detachment percentage, calculate SCORTEN, and intensify supportive planning. [18] |
| Conjunctival symptoms, visual complaints, or ocular-surface disease | Signals risk for acute and chronic ocular morbidity. [5][21] | Obtain urgent ophthalmology assessment and initiate ocular lubrication. [21] |

## Identify and document the culprit medication

The most consequential cause-directed intervention is permanent avoidance of the responsible drug.

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. [5][1]

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. [5][8][10][12]

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. [18][20]

Use pharmacogenetic prevention prospectively in relevant new-drug starts rather than as an acute diagnostic test. Clinically implemented markers include HLA-B*15:02 and HLA-A*31:01 for new carbamazepine users, HLA-B*58:01 for allopurinol, and HLA-B*57:01 for abacavir. [24]
- Specifically ask about carbamazepine, phenytoin, lamotrigine, oxcarbazepine, allopurinol, trimethoprim-sulfamethoxazole and other sulfonamide anti-infectives, oxicam NSAIDs, and nevirapine. [5][18][23]
- Do not restart a medication merely because another potential trigger exists; retain all plausible culprit drugs on the avoidance list until specialist-led causality assessment is complete. [1][20]
- For a future carbamazepine decision, incorporate HLA-B*15:02 and HLA-A*31:01 testing in populations and clinical contexts for which pharmacogenetic implementation is applicable. [24]

*High-priority culprit-drug and prevention considerations. [5][18][23][24]*

| Drug or class | SJS/TEN relevance | Action |
| --- | --- | --- |
| Carbamazepine and related aromatic antiseizure medications | Carbamazepine, phenytoin, lamotrigine, and oxcarbazepine are repeatedly implicated; HLA-B*15:02 is associated with risk across aromatic antiseizure drugs. [5][23] | Stop during suspected SJS/TEN; consider HLA-B*15:02 and HLA-A*31:01 testing before new carbamazepine use when applicable. [24] |
| Allopurinol | A high-risk drug associated with SJS/TEN; HLA-B*58:01 has a strong pharmacogenetic association. [18][23] | Stop during suspected SJS/TEN; use HLA-B*58:01 testing prospectively for prevention where clinically indicated. [24] |
| Sulfonamide anti-infectives | Anti-infective sulfonamides are high-risk medications for SJS/TEN. [18] | Stop promptly when temporally plausible and document avoidance if implicated. [18][20] |
| Abacavir | Severe cutaneous adverse-reaction prevention has been implemented through HLA-B*57:01 testing. [24] | Use prospective HLA-B*57:01 testing before treatment initiation. [24] |

## Deliver organ-specific supportive care and prevent early complications

Supportive management is the acute treatment foundation regardless of adjunctive immunomodulator selection.

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. [4][21]

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. [21]

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. [11][19]

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. [5]
- Perform ongoing respiratory assessment and escalate respiratory support when mucosal disease or clinical deterioration warrants it. [4]
- Reassess oral intake and nutrition daily; nutritional support is a core component of acute care. [4][21]
- Use local wound care and protect denuded epidermis while coordinating management with clinicians experienced in burn or intensive care. [4][14]

*Organ-directed supportive-care priorities in acute SJS/TEN. [4][5][19][21]*

| Domain | Immediate action | Monitoring target |
| --- | --- | --- |
| Skin and fluid balance | Provide fluid replacement and local wound treatment. [4] | Progression of detachment, wound status, and clinical volume status. [4][21] |
| Nutrition and oral mucosa | Provide nutritional support when oral disease limits intake. [4][21] | Ability to maintain intake and need for continuing nutrition support. [4] |
| Respiratory tract | Assess for respiratory involvement and provide respiratory management when needed. [4] | Clinical respiratory deterioration requiring escalation. [4] |
| Eyes | Use lubricating ointment and obtain ophthalmology consultation for ocular involvement. [21] | Acute ocular severity and chronic visual complications. [5][20] |
| Infection | Do not default to systemic prophylactic antibiotics; treat clinical sepsis. [21] | Clinical signs and symptoms of sepsis. [21] |

## Choose systemic immunomodulatory therapy cautiously

Adjunctive systemic treatment remains variable; supportive care and culprit-drug cessation cannot be deferred.

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. [4][20]

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. [2][7][20]

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. [2][6][19]

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. [4][13]
- Discuss systemic therapy with dermatology and the receiving critical-care or burn team rather than applying a uniform regimen. [14][20]
- Use renal function as a practical selection constraint when considering cyclosporine because renal impairment is a relevant underlying condition in treatment literature. [7]
- Avoid thalidomide: the only randomized controlled trial cited in this literature found increased mortality. [2]

*Systemic treatments reported in SJS/TEN and limits of the evidence. [2][4][6][13][19][20]*

| Intervention | Reported regimen or finding | Practice implication |
| --- | --- | --- |
| Cyclosporine | A retrospective comparison used 3–5 mg/kg/day for up to 7 days and observed lower-than-SCORTEN-predicted mortality in the cyclosporine group. [2] | Potential adjunct in experienced-center protocols; evidence remains nondefinitive and renal impairment is a relevant consideration. [7][20] |
| IVIG | A retrospective comparison used 1 g/kg/day for 3 days; prospective and comparative reports have not shown consistent benefit. [2][6] | Do not assume benefit or substitute IVIG for supportive care. [4][20] |
| Systemic corticosteroids | Used in clinical practice, but effectiveness remains uncertain and controversy persists. [4][21] | Consider only within an experienced multidisciplinary treatment strategy. [4][20] |
| Etanercept | Used alone or with systemic corticosteroids in observational studies. [5][13] | No definitive consensus regimen; use should be protocol- and specialist-directed. [20] |
| Plasma exchange | Recent data did not show benefit over IVIG among patients not responding effectively to corticosteroids. [4] | Reserve for selected specialist-directed circumstances rather than routine use. [4] |
| Thalidomide | The cited randomized trial found increased mortality. [2] | Do not use for SJS/TEN. [2] |

## Prevent re-exposure and organize postacute surveillance

Discharge is appropriate only after the medication-avoidance plan and mucosal follow-up plan are explicit.

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. [18][20]

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. [5][11]

For future high-risk prescribing, use established pharmacogenetic prevention pathways before initiating selected drugs: HLA-B*15:02 and HLA-A*31:01 for carbamazepine, HLA-B*58:01 for allopurinol, and HLA-B*57:01 for abacavir. These tests are prevention tools for new treatment decisions, not proof of the culprit in an acute episode. [24]
- Include the culprit drug, onset date, clinical phenotype, mucosal sites involved, biopsy result when obtained, and hospitalization severity in the discharge record. [17][18]
- Ensure follow-up addresses vision and ocular-surface symptoms even after skin healing. [5][20]
- Review future anticonvulsant, allopurinol, and antiretroviral decisions through a pharmacogenetic and allergy-avoidance lens. [23][24]

*Discharge actions that reduce avoidable morbidity after SJS/TEN. [5][17][20][24]*

| Action | Documentation or referral | Purpose |
| --- | --- | --- |
| Culprit-drug avoidance | Record suspected or confirmed SJS/TEN and implicated medication in the allergy list; give written avoidance instructions. [18][20] | Prevent inadvertent re-exposure. [18] |
| Ophthalmic surveillance | Schedule ophthalmology follow-up after acute ocular disease. [5][20] | Detect and manage chronic ocular complications, including vision-threatening disease. [5] |
| Site-specific sequelae follow-up | Coordinate care for respiratory, reproductive, and mental health sequelae when those systems were involved. [5][11] | Address chronic morbidity beyond skin healing. [5] |
| Future pharmacogenetic testing | Use HLA-B*15:02/HLA-A*31:01, HLA-B*58:01, or HLA-B*57:01 testing before indicated new drug starts. [24] | Prevent selected severe cutaneous adverse reactions. [24] |

## References
1. [PDF] Scientific Review for Proposed Administrative Order OTC000035 — www.accessdata.fda.gov — https://www.accessdata.fda.gov/drugsatfda_docs/omuf/General/Scientific%20Review%20for%20Proposed%20Administrative%20Order%20OTC000035%20-%20Amending%20OTCM013_06.14.2024.pdf
2. New Treatment for Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis? | NEJM Clinician — clinician.nejm.org — https://clinician.nejm.org/new-treatment-stevens-johnson-syndrome-toxic-epidermal-necrolysis-nejm-jw.NA35627
3. Economic Evaluation of HLA-B*15:02 Genotyping for Asian ... — jamanetwork.com — https://jamanetwork.com/journals/jamadermatology/fullarticle/2818474
4. Effectiveness of early treatment with plasma exchange in patients with Stevens–Johnson syndrome and toxic epidermal necrolysis | Scientific Reports — www.nature.com — https://www.nature.com/articles/s41598-024-53653-5
5. Multiomic single-cell sequencing defines tissue-specific responses in Stevens-Johnson syndrome and toxic epidermal necrolysis | Nature Communications — www.nature.com — https://www.nature.com/articles/s41467-024-52990-3
6. High-dose intravenous immunoglobulin in the treatment of toxic epidermal necrolysis: a study of ocular benefits | Eye — www.nature.com — https://www.nature.com/articles/6701653
7. Recent progress in Stevens–Johnson syndrome/toxic epidermal ... — academic.oup.com — https://academic.oup.com/bjd/article-pdf/doi/10.1093/bjd/ljae321/61052287/ljae321.pdf
8. Guidelines for the Management of Stevens–Johnson Syndrome and ... — onlinelibrary.wiley.com — https://onlinelibrary.wiley.com/doi/10.1111/1346-8138.70136
9. Toxic epidermal necrolysis: the past, the guidelines and challenges ... — onlinelibrary.wiley.com — https://onlinelibrary.wiley.com/doi/pdf/10.1111/bjd.14682
10. Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: A ... — onlinelibrary.wiley.com — https://onlinelibrary.wiley.com/doi/10.1111/ajd.70078?af=R
11. Diagnosis and Management of Stevens‐Johnson ... — onlinelibrary.wiley.com — https://onlinelibrary.wiley.com/doi/full/10.1111/pde.70149
12. The Medication Risk of Stevens–Johnson Syndrome and Toxic ... — ascpt.onlinelibrary.wiley.com — https://ascpt.onlinelibrary.wiley.com/doi/10.1002/cpt.1071
13. Evaluation of Combination Therapy With Etanercept and Systemic Corticosteroids for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Multicenter Observational Study - ScienceDirect — www.sciencedirect.com — https://www.sciencedirect.com/science/article/abs/pii/S2213219822001180
14. Society of Dermatology Hospitalists supportive care guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults - ScienceDirect — www.sciencedirect.com — https://www.sciencedirect.com/science/article/abs/pii/S0190962220303121
15. The effects of systemic cyclosporine in acute Stevens-Johnson syndrome/toxic epidermal necrolysis on ocular disease - ScienceDirect — www.sciencedirect.com — https://www.sciencedirect.com/science/article/abs/pii/S1542012420300811
16. Burn unit care of Stevens Johnson syndrome/toxic epidermal necrolysis: A survey - ScienceDirect — www.sciencedirect.com — https://www.sciencedirect.com/science/article/abs/pii/S0305417915003915
17. Toxic epidermal necrolysis and Stevens-Johnson syndrome - PubMed — www.ccjm.org — https://www.ccjm.org/lookup/external-ref?access_num=21162721&link_type=MED&atom=%2Fccjom%2F92%2F6%2F331.atom
18. Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis - PubMed — www.ncbi.nlm.nih.gov — https://www.ncbi.nlm.nih.gov/pubmed/29188475
19. Retrospective Study of Patients With SJS/TEN Treated at a Tertiary Burn Unit in Canada: Overview of 17 Years of Treatment - PubMed — www.ncbi.nlm.nih.gov — http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=33393825
20. The Effects of Systemic Cyclosporine in Acute Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis on Ocular Disease - PMC — www.ncbi.nlm.nih.gov — https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7811361
21. [PDF] Influenza B virus infection and Stevens–Johnson syndrome — stacks.cdc.gov — https://stacks.cdc.gov/view/cdc/79867/cdc_79867_DS1.pdf
22. A Retrospective Cohort Study of the Management and Outcomes of ... — www.jaci-inpractice.org — https://www.jaci-inpractice.org/article/S2213-2198(18)30346-5/abstract
23. Current Pharmacogenetic Perspective on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis — pmc.ncbi.nlm.nih.gov — https://pmc.ncbi.nlm.nih.gov/articles/PMC8107822
24. Pharmacogenetic Testing for Prevention of Severe Cutaneous Adverse Drug Reactions — pmc.ncbi.nlm.nih.gov — https://pmc.ncbi.nlm.nih.gov/articles/PMC7346738

## Editorial note

Prepared from cited clinical literature using Astra's research workflow. Verify recommendations against current guidance and patient-specific factors.