Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis is not the name you expected.
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.
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Stevens Johnson Syndrome Foundation
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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 www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
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Last Updated: 7/14/2015
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