The following information may help to address your question:
What is 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.
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. Having a gene
called HLA-B 1502
, increases risk of having Stevens-Johnson syndrome.
It is an emergency medical condition that usually requires hospitalization. Treatment focuses on eliminating the underlying cause, controlling symptoms and minimizing complications and includes pain medication to reduce discomfort, medication to relieve itching (antihistamines), antibiotics
to control infection, when needed and medication to reduce skin inflammation (topical steroids).
Last updated: 7/8/2015
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.
To be classified as Stevens-Johnson syndrome, the condition must involve less than 10% of the body surface area.
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
, 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.
Last updated: 6/11/2013
What causes Stevens-Johnson syndrome?
Stevens-Johnson syndrome is usually caused by an allergic reaction to a drug or medication.
The most common medications to trigger the condition are antibiotics, followed by analgesics (pain relievers), cough and cold medications, NSAIDs, anticonvulsants, and anti-gout drugs. Infections can also cause Stevens-Johnson syndrome. Viral infections that may precede the condition include herpes (simplex or zoster), pneumonia, HIV, and hepatitis.
In some cases, the underlying cause is unknown (idiopathic).
Some people with Stevens-Johnson syndrome have a genetic predisposition
which increases their risk of developing the condition in response to triggering factors such as medications. The genetic variation
most strongly associated with Stevens-Johnson syndrome occurs in the HLA-B
This gene is part of a family of genes called the human leukocyte antigen (HLA) complex
. This complex of genes helps the immune system
distinguish the body's own proteins
from proteins made by foreign invaders (such as viruses
Variations in several other HLA and non-HLA genes have also been studies as possible risk factors
for Stevens-Johnson syndrome.
However, most people with the genetic variations that increase the risk for Stevens-Johnson syndrome never develop the disease, even if they are exposed to drugs that can trigger it. It is likely that there are other factors involved in this complex process which determines whether a person ultimately develops the condition.
Last updated: 9/6/2016
What complications may be associated with Stevens-Johnson syndrome?
Possible complications of Stevens-Johnson syndrome include:
- 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: 9/6/2016
How might Stevens-Johnson syndrome be treated?
Stevens-Johnson syndrome may be difficult to treat. Patients should be admitted to an intensive care or burn unit as soon as the diagnosis is suspected.
Treatment of severe symptoms may include:
- 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.
Last updated: 6/12/2013
What is the prognosis for individuals with Stevens-Johnson syndrome?
Although regrowth of the skin following Stevens-Johnson syndrome (SJS) is rapid (2-3 weeks), recovery can take weeks to months, depending on the severity of symptoms. Feelings of overwhelming tiredness may persist for months. Depression may also develop. For some, chronic complications may develop within weeks to months of an acute episode and can severely affect quality of life.
Recurrence of SJS has been documented, though the actual recurrence risk is debated. Estimates of recurrence range from 3-7% for adults and 7-18% for children. Studies suggest recurrence risk is higher when SJS is associated with an infection or unknown cause. Recurrence in SJS associated with a medication reaction is believed to be rare, however can occur if the person is given the same or closely related triggering medication.
In some patients, SJS can be fatal.
Last updated: 6/6/2017
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