Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis

Stevens Johnson Syndrome is an immune complex hypersensitivity reaction that can be caused from an infection or immune response to drugs. It is a severe expression of a simple rash known as erythema multiforme. SJS is also known as erythema multiforme major. It affects all ages and genders including pediatric populations. The most severe form of SJS is toxic epidermal necrolysis (TENS). SJS occurs twice as often in men as in women. Most cases of SJS appear in children and young adults under age 30. Females with SJS are twice as likely as males to develop TENS, and have an even higher chance if taking a category of drugs known as NSAIDs, non-steroidal anti-inflammatory agents.

Erythema multiforme minor

• A rash that is usually caused by an immune response to drugs or to an infection
• Appears on the skin in "multiform" ways including macules, blisters, hives, and target appearing lesions.
• May involve the palms of hands, soles of the feet, mucous membranes, face and extremities.
• Usually is self-limited
• Occurs in 10.2 to 46.5 cases per million per year.

Stevens-Johnson Syndrome (SJS)

• Also known as Erythema multiform major
• Most severe and occasionally fatal variant of erythema multiform. The rash is widespread and severe and involves the eyes, mouth, vaginal, urethral, gastrointestinal, respiratory tract and other internal organs. It appears as target like lesions.
• Begins with symptoms of non-specific upper respiratory tract infection
• Produces fevers
• First 1 to 14 days symptoms of fever, sore throat, chills, headache, malaise, and usually vomiting and diarrhea.
• Skin loss may lead to dehydration, infection or death
• Ocular involvement can include severe conjunctivitis, iritis, conjunctiva and corneal abrasions and corneal perforation.
• Occurs in slightly more than 1 to 7 cases per million per year.

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