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Genetic and Rare Diseases Information Center (GARD)

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Stevens-Johnson syndrome


Other Names for this Disease
  • Erythema multiforme major
  • SJS
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I have a friend whose grandson recently died from complications associated with Stevens-Johnson syndrome. I have not been able to find information about this condition. What information can you provide?

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What is Stevens-Johnson syndrome?

Stevens-Johnson Syndrome (SJS), also called erythema multiforme major, is a limited form of toxic epidermal necrolysis. This disorder affects the skin, mucous membranes and eyes.[1][2] Stevens-Johnson syndrome occurs twice as often in men as women, and most cases appear in children and young adults under 30, although it can develop in people at any age.[1] It is an emergency medical condition that usually requires hospitalization. Treatment focuses on eliminating the underlying cause, controlling symptoms and minimizing complications.[3]
Last updated: 11/22/2010

What are the signs and symptoms of Stevens-Johnson syndrome?

Often, Stevens-Johnson syndrome begins with flu-like symptoms, followed by a painful red or purplish rash that spreads and blisters, eventually causing the top layer of the skin to die and shed.[3] To be classified as Stevens-Johnson syndrome, the condition must involve less than 10% of the body surface area.[2] The condition is characterized by painful, blistery lesions on the skin and the mucous membranes (the thin, moist tissues that line body cavities) of the mouth, throat, genital region, and eyelids. It can also cause serious eye problems, such as severe conjunctivitis; iritis, an inflammation inside the eye; corneal blisters and erosions; and corneal holes. In some cases, the ocular complications from this condition can be disabling and lead to severe vision loss.[1]
Last updated: 6/11/2013

What causes Stevens-Johnson syndrome?

The exact cause of Stevens-Johnson syndrome is unknown in 25 to 30% of cases.[1][2][3][4] In those cases in which the cause can be determined, it is believed to be related to an adverse allergic drug reaction. Almost any drug--but most particularly sulfa drugs--can cause Stevens-Johnson syndrome. The allergic reaction to the drug may not occur until 7-14 days after first using it. Stevens-Johnson syndrome can also be preceded by a viral infection, such as herpes or the mumps.[1] In rare cases, Stevens-Johnson syndrome may be caused by an illness or bone marrow transplantation.[2][3]
Last updated: 6/11/2013

What complications may be associated with Stevens-Johnson syndrome?

Possible complications of Stevens-Johnson syndrome include:[3]

  • Secondary skin infections (cellulitis) - which can lead to life-threatening complications like meningitis and sepsis  
  • Sepsis - which occurs when bacteria from an infection enters the bloodstream and spreads throughout the body, possibly leading to shock and organ failure
  • Eye Problems - which may include extensive tissue damage and scarring which may result in blindness
  • Damage to internal organs - which may involve the lungs, heart, kidney and liver
  • Permanent skin damage - which may include abnormal bumps and pigmentation as well as hair loss and abnormal growth of the finger and toe nails
Last updated: 3/15/2010

How might Stevens-Johnson syndrome be treated?

Stevens-Johnson syndrome may be difficult to treat.[4] Patients should be admitted to an intensive care or burn unit as soon as the diagnosis is suspected.[2][4]

Treatment of severe symptoms may include:[4]

  • Antibiotics to control any skin infections
  • Corticosteroids to control inflammation
  • Intravenous immunoglobulins (IVIG) to stop the disease process

Treatment for the eye may include artificial tears, antibiotics, or corticosteroids.[1]

Last updated: 6/12/2013

What is the prognosis for individuals with Stevens-Johnson syndrome?

Although regrowth of the skin following Stevens-Johnson syndrome is rapid (2-3 weeks), recovery can take weeks to months, depending on the severity of the condition.[2][3]  About one-third of all patients diagnosed with Stevens-Johnson syndrome have recurrences of the disease.[1] In some patients, the diseases is fatal.[4]
Last updated: 3/15/2010

References