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Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: Diagnosis and Management in the Emergency Department
What ED Clinicians Need to Know
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but potentially fatal mucocutaneous emergencies that exist on a spectrum and are most often triggered by a medication reaction. Because early presentations can mimic far more common, benign rashes, SJS/TEN epitomizes a high-stakes diagnostic challenge in the ED. This issue focuses on rapid recognition, distinguishing SJS/TEN from other dermatologic conditions, and the priorities for initial stabilization and supportive care from arrival through disposition.
Successful ED management hinges on a few critical decision-making steps: recognizing the condition early, identifying and discontinuing the causative agent, and delivering supportive care that broadly follows burn-care principles. Validated prognostic tools help stratify risk and guide the intensity of care, while early multidisciplinary involvement and timely transfer support better outcomes. The issue also highlights common pitfalls that can delay diagnosis and worsen outcomes.
Why This Topic Matters in the ED
- Dermatologic complaints are common in the ED, and SJS/TEN represents the kind of rare, rapidly progressive presentation that carries substantial morbidity and mortality if missed.
- Early SJS/TEN can look like a benign viral rash or drug eruption, making prompt recognition an important skill for emergency clinicians.
- Because most cases are medication-related, a careful medication history and prompt discontinuation of the offending agent can directly influence outcomes.
- Timely supportive care, specialist involvement, and transfer decisions are central to reducing complications such as infection, fluid loss, and long-term ocular damage.
Clinical Content at a Glance
Clinical Q&A
- What distinguishes SJS/TEN from other dermatologic emergencies?
- A painful rash with mucous membrane involvement and a positive Nikolsky sign are hallmark features. Mucosal involvement in particular helps separate SJS/TEN from more benign rashes.
- What most commonly triggers SJS/TEN?
- The majority of cases are caused by a medication reaction, though infections and other factors can contribute. Identifying and stopping the offending agent is a central priority.
- How should clinicians prioritize initial management?
- Key priorities include recognizing the condition early, discontinuing the suspected causative agent, providing supportive care, and engaging the appropriate specialists. Management broadly follows burn-care principles.
- What role do prognostic scoring tools play?
- Validated tools such as SCORTEN and ABCD-10 help stratify mortality risk and inform the intensity of supportive care and disposition decisions.
- When is escalation to an ICU or burn center considered?
- Escalation is considered for patients with extensive skin involvement, airway or hemodynamic compromise, or other signs of severe disease. Early transfer has been associated with improved outcomes.
Case Snapshots
Case 1 — Allopurinol-Associated Reaction: A 60-year-old man develops fever, sore throat, and a rapidly spreading, painful, blistering rash weeks after starting allopurinol for gout. The issue follows recognition, withdrawal of the offending agent, supportive care, and transfer for definitive management.
Case 2 — Antiseizure Medication Reaction: A 22-year-old woman with epilepsy presents with oral ulcers, eye involvement, and Nikolsky-positive lesions after a recent increase in her lamotrigine dose. The issue illustrates the diagnostic approach, risk stratification, and multidisciplinary admission decision-making.
Case 3 — Pediatric SJS/TEN After Infection: An 8-year-old girl develops fever, conjunctivitis, and a blistering rash following a recent mycoplasma infection. The issue explores how recognition and management considerations can differ in pediatric patients.
Clinical Tools
- Table of inciting factors associated with SJS/TEN (medications, infections, and other contributors)
- Differential diagnosis organized by cutaneous and mucosal rash morphology
- SCORTEN and ABCD-10 prognostic scoring tools with predicted mortality
- Wallace Rule of Nines for body surface area assessment
- Clinical images of characteristic skin, oral, and ocular findings
- Clinical pathway for the evaluation and management of suspected SJS/TEN in the ED
Risk Management Pitfalls
- Mistaking early SJS/TEN for a benign viral rash or drug eruption
- Overlooking a recently started or recently dose-adjusted medication as the trigger
- Skipping a full mucosal examination of the eyes, mouth, and genitals
- Delaying ophthalmology consultation in patients with suspected SJS/TEN
- Underestimating disease severity and fluid needs, leading to delayed admission or escalation of care
Key References
Following are the most informative references cited in this paper, as determined by the authors.
6. * Dodiuk-Gad RP, Chung WH, Valeyrie-Allanore L, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: an update. Am J Clin Dermatol. 2015;16(6):475-493. (Review) DOI: 10.1007/s40257-015-0158-0
12. * Seminario-Vidal L, Kroshinsky D, Malachowski SJ, et al. Society of Dermatology Hospitalists supportive care guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults. J Am Acad Dermatol. 2020;82(6):1553-1567. (Clinical practice guidelines) DOI: 10.1016/j.jaad.2020.02.066
32. * Marks ME, Botta RK, Abe R, et al. Updates in SJS/TEN: collaboration, innovation, and community. Front Med (Lausanne). 2023;10:1213889. (Review) DOI: 10.3389/fmed.2023.1213889
38. * Micheletti RG, Chiesa-Fuxench Z, Noe MH, et al. Stevens-Johnson syndrome/toxic epidermal necrolysis: a multicenter retrospective study of 377 adult patients from the United States. J Invest Dermatol. 2018;138(11):2315-2321. (Multicenter retrospective cohort study; 377 patients) DOI: 10.1016/j.jid.2018.04.027
41. * Jacobsen A, Olabi B, Langley A, et al. Systemic interventions for treatment of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome. Cochrane Database Syst Rev. 2022;3(3):CD013130. (Cochrane review; 9 studies, 308 patients) DOI: 10.1002/14651858.CD013130.pub2
CME Information
- 4 CME credits (available for subscribers)
- Includes Pharmacology
- View complete CME Information here
