Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

National Organization for Rare Disorders, Inc.

Skip to the navigation

It is possible that the main title of the report Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis is not the name you expected.

Disorder Subdivisions

  • None

General Discussion

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) represent opposite ends of a spectrum of disease that results from an adverse reaction, most often to certain medications. SJS is the less severe end, but still represents a serious condition and potential medical emergency. TEN is a severe, life-threatening disorder. These disorders are differentiated by the degree of skin detachment. The consensus definition published in 1993 states that SJS affects less than 10% of the body surface area; TEN affects more than 30% of the body surface area. The term SJS/TEN-overlap syndrome is used to describe cases in which 10%-30% of the body surface area is detached. The reaction may start with a persistent fever and nonspecific, flu-like symptoms followed by appearance of erythematous macules (red spots) that may cover a large part of the body, and painful blistering of the skin and mucous membranes. The eyes are often involved. Numerous drugs have been reported to cause SJS and TEN and the following have shown an increased risk in larger studies: antibacterial sulfonamides, non-steroidal anti-inflammatory drugs of the oxicam type, certain anti-seizure drugs (antiepileptics), allopurinol and nevirapine. However, approximately one quarter (25%) of cases are not caused by drugs, but potentially by infections or have to be considered as idiopathic (of unknown cause).
Individuals suspected of SJS or TEN should immediately stop taking the offending drug if it is known and all nonessential medications if it is not. Prompt recognition and early treatment are essential. It is also important to note that these disorders represent a spectrum of disease ranging from mild cases to those with severe, life-threatening complications. Consequently, every case is unique and the description of symptoms below will not apply to all individuals.

SJS and TEN are classified as severe cutaneous adverse reactions (SCAR), a subcategory of adverse drug reactions (ADR). Unlike individuals with SJS and TEN, most individuals with a reactive skin disease have a mild and self-limiting condition. For years, confusing and contradictory terminology has been used to describe these disorders and controversy still exists as to the best way to classify them. In the past, erythema multiforme (EM) was considered part of this disease spectrum, but is now considered a distinct disorder. NORD has a separate report on erythema multiforme. These disorders are generally broken down into SJS, SJS/TEN overlap, and TEN. SJS was first described in the medical literature in 1922 by doctors A.M. Stevens and F.C. Johnson. The term toxic epidermal necrolysis was introduced in the medical literature in 1956 by Dr. A. Lyell and is also known as Lyell syndrome.

Supporting Organizations

Genetic and Rare Diseases (GARD) Information Center

PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311

NIH/National Institute of Allergy and Infectious Diseases

NIAID Office of Communications and Government Relations
5601 Fishers Lane, MSC 9806
Bethesda, MD 20892-9806
Tel: (301)496-5717
Fax: (301)402-3573
Tel: (866)284-4107

NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

Information Clearinghouse
One AMS Circle
Bethesda, MD 20892-3675
Tel: (301)495-4484
Fax: (301)718-6366
Tel: (877)226-4267

Ocular Immunology and Uveitis Foundation

5 Cambridge Center
8th Floor
Cambridge, MA 2142
Tel: (617)621-6377
Fax: (781)431-2042
Tel: (866)353-6377

Stevens Johnson Syndrome Foundation

PO Box 350333
Westminster, CO 80035-0333
Tel: (303)635-1241
Fax: (303)648-6686

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

Last Updated:  7/14/2015
Copyright  2015 National Organization for Rare Disorders, Inc.