Stevens-Johnson syndrome and toxic epidermal necrolysis

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Last reviewed: 8 Apr 2025

Last updated: 07 Feb 2025

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe skin detachments with mucocutaneous complications associated with a history of medication use, most commonly anticonvulsants, antibiotics, and nonsteroidal anti-inflammatory drugs.

Patients with HIV or active cancer are at an increased risk of SJS/TEN.

SJS/TEN may present with mild symptoms in early disease, but the condition progresses over several days; close monitoring and regular reassessment is essential.

Once a diagnosis is established, the causative agent should be identified and withdrawn; the patient should receive skin care and supportive management.

Transfer to a burn center, a specialized wound care center, or a dermatology intensive care unit is recommended for patients diagnosed with SJS/TEN.

Patients at risk may need human leukocyte antigen screening if they are to receive medications such as carbamazepine, allopurinol, or trimethoprim/sulfamethoxazole.

Definition

SJS is a severe skin detachment with mucocutaneous complications.[1] It is an immune reaction to foreign antigens. SJS is a less severe manifestation of TEN.[2]

Classification is dependent on the percentage of skin involvement: SJS has <10% total body surface area (TBSA) involvement; SJS/TEN overlap has 10% to 30% TBSA involvement; and TEN has >30% TBSA involvement.[3]

History and exam

Key diagnostic factors

rash
mucosal involvement
Nikolsky sign
blisters

Full details

Risk factors

patients with active cancer
anticonvulsant medications
recent infection
recent antibiotic use
other medications
systemic lupus erythematosus
HIV-positive
radiation therapy
human leukocyte antigen and genetic predisposition
smallpox vaccination
bone marrow transplantation

Full details

Diagnostic tests

1st tests to order

skin biopsy
blood cultures
complete blood count
glucose
magnesium
phosphate
blood urea nitrogen
bicarbonate
serum electrolytes
serum creatinine
liver function tests
C-reactive protein (CRP)
arterial blood gases and saturation of oxygen
chest x-ray
coagulation studies
skin swab from lesional skin
Mycoplasma serology

Full details

Tests to avoid

erythrocyte sedimentation rate (ESR)

Full details

Tests to consider

direct immunofluorescence

Full details

Treatment algorithm

ACUTE

all patients

Contributors

Authors

Areta Kowal-Vern, MD

Adjunct Research Faculty

Arizona Burn Center

Valleywise Health Medical Center

Phoenix

Disclosures

AK-V performs occasional manuscript reviews on various topics for Research Square, Inc. and the Journal of Burn Care and Research in the US for which she receives a nominal monetary fee. AK-V is an author of a reference cited in this topic.

Peer reviewers

Barbara A. Latenser, MD

Director Burn Unit

Department of Surgery

University of Iowa Hospitals

Iowa City

Disclosures

BAL declares that she has no competing interests.

Tina Palmieri, MD

Assistant Chief of Burns

Shriners Hospital

UC Davis

Sacramento

Disclosures

TP declares that she has no competing interests.

Venkat Gudi, MB BS

Consultant Dermatologist

Department of Dermatology

West Suffolk Hospital

Bury St. Edmunds

Disclosures

VG declares that he has no competing interests.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Bastuji-Garin S, Rzany B, Stern RS, et al. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol. 1993 Jan;129(1):92-6. Abstract

Creamer D, Walsh SA, Dziewulski P, et al. UK guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults 2016. Br J Dermatol. 2016 Jun;174(6):1194-227.Full text Abstract

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 Jun;82(6):1553-67. Abstract

Gregory DG. New grading system and treatment guidelines for the acute ocular manifestations of Stevens-Johnson syndrome. Ophthalmology. 2016 Aug;123(8):1653-8. Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

Differentials
- Drug rash with eosinophilia and systemic symptoms (DRESS)
- Staphylococcal scalded skin syndrome
- Toxic shock syndrome
More Differentials
Guidelines
- Supportive care guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults
- Management of Stevens-Johnson syndrome/toxic epidermal necrolysis in children and young people (0 to 17 years)
More Guidelines
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Stevens-Johnson syndrome and toxic epidermal necrolysis

Summary

Definition

History and exam

Key diagnostic factors

Risk factors

Diagnostic tests

1st tests to order

Tests to avoid

Tests to consider

Treatment algorithm

all patients

Contributors

Authors

Areta Kowal-Vern, MD

Disclosures

Peer reviewers

Barbara A. Latenser, MD

Disclosures

Tina Palmieri, MD

Disclosures

Venkat Gudi, MB BS

Disclosures

References

Key articles

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

Differentials

Guidelines

Log in or subscribe to access all of BMJ Best Practice

Log in or subscribe to access all of BMJ Best Practice

Log in to access all of BMJ Best Practice