Stevens–Johnson syndrome

Stevens–Johnson syndrome
Synonyms	erythema multiforme major
Man with Stevens–Johnson syndrome
Classification and external resources
Specialty	Dermatology
ICD-10	L51.1
ICD-9-CM	695.13
OMIM	608579
DiseasesDB	4450
MedlinePlus	000851
eMedicine	emerg/555 derm/405
Patient UK	Stevens–Johnson syndrome
MeSH	D013262
Orphanet	36426
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Stevens–Johnson syndrome, a form of toxic epidermal necrolysis, is a life-threatening skin condition, in which cell death causes the epidermis to separate from the dermis. The syndrome is thought to be a hypersensitivity complex that affects the skin and the mucous membranes. The best known causes are certain medications (such as lamotrigine), but it can also be due to infections, or more rarely, cancers.

Stevens–Johnson syndrome (SJS) is a milder form of toxic epidermal necrolysis (TEN). These conditions were first recognised in 1922. A classification first published in 1993, that has been adopted as a consensus definition, identifies Stevens–Johnson syndrome, toxic epidermal necrolysis, and SJS/TEN overlap. All three are part of a spectrum of severe cutaneous reactions (SCAR) which affect skin and mucous membranes. The distinction between SJS, SJS/TEN overlap, and TEN is based on the type of lesions and the amount of the body surface area with blisters and erosions. Blisters and erosions cover between 3% and 10% of the body in SJS, 11–30% in SJS/TEN overlap, and over 30% in TEN. The skin pattern most commonly associated with SJS is widespread, often joined or touching (confluent), papuric spots (macules) or flat small blisters or large blisters which may also join together. These occur primarily on the torso.

SJS, TEN, and SJS/TEN overlap can be mistaken for erythema multiforme. Erythema multiforme, which is also within the SCAR spectrum, differs in clinical pattern and etiology. Although both SJS and TEN can also be caused by infections, they are most often adverse effects of medications.

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