Stevens-Johnson Syndrome

by William L. Weston, M.D.
Professor of Dermatology
University of Colorado Health Sciences Center

What is SJS?

SJS, or Stevens-Johnson Syndrome, is an abrupt, severe injury to the mouth, eyes,
and skin, where large sheets of mucosa or skin are destroyed and then shed. The
occurrence of SJS is uncommon.

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What causes SJS?

For most individuals, SJS is the result of a drug reaction. Sulfa drugs, seizure
drugs, and analgesics (pain relievers) are the most common medicines to cause SJS;
however, a large number of drugs can cause it. In a few individuals, infections,
such as pneumonias caused by Mycoplasma, may cause SJS.

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Who gets SJS?

SJS mostly occurs in toddlers and in young children.

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How does a drug reaction cause disease?

The skin of the person who has a reaction to a drug may not correctly eliminate
it. The reaction most likely occurs because of a genetic mutation in one of the
enzymes that is responsible for eliminating drugs from the body. The drug builds
up in the lining of the skin, mouth, and eyes, and severely damages the tissue.
It is similar to a burn, but the damage occurs from the inside out. Internal organs
also may be involved.

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What are the common findings?

The initial signs of SJS are bloody crusts on the lips, a sore mouth that has a
foul smell, and purple-red tender spots on the skin. Drinking and eating are difficult,
and light is painful to the eyes. Large areas of tissue death occur, and large blisters
may form, followed by a loss of large sheets of the skin or the mouth. The linings
of the eyes have pus drainage, and they may heal with scarring so that the eyelids
do not move normally. The cornea also may erode. Fingernails may be shed, and a
loss of skin color may occur. Severe stomach problems may occur in some individuals,
and diarrhea and kidney or liver damage may result. The internal lining of the airway
may slough off, blocking breathing.

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How is SJS diagnosed?

Most physicians diagnose SJS from the involvement of the mouth and the eyes, plus
the appearance of skin lesions. Sometimes, a skin biopsy may be needed to distinguish
SJS from other conditions, such as pemphigus, which is another blistering condition.

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How is SJS treated?

There is not a specific treatment for SJS at the time of an attack. Treatment for
SJS consists of replacing fluids, calories, and salts, and then treating the skin
as if it was a burn. If a drug is suspected of causing SJS, it should be stopped.

SJS is a severe, life-threatening condition, and the best treatment occurs when
the child is admitted to a hospital with a pediatric burn unit or a pediatric intensive
care unit.

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What are the complications?

The complications of SJS are similar to a severe burn. The following complications
may occur: infection through the open skin, dehydration, salt disturbances, fever,
scarring, fingernail loss, loss of skin color, breathing problems, pneumonia, kidney
failure, liver problems, and death.

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How can SJS be prevented?

SJS may be prevented by avoiding those drugs that have caused reactions in the past,
and by not taking those drugs that are more likely to cause SJS. However, for most
individuals, SJS appears unexpectedly and cannot be prevented.

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What research is being done?

Researchers are currently examining treatments that block the cell death pathways.
Potential genetic mutations also are being examined, which may help in developing
tests that can predict who will get a severe reaction to a particular class of drugs.

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About the Author

Dr. Weston is a Professor of Pediatrics and Dermatology at the University of Colorado
Health Sciences Center and Chair of the Department of Dermatology. His scientific
and clinical interests include Cutaneous immunology, Cutaneous virology, and Pediatric
Dermatology.

Dr. Weston is the primary author of the Color Textbook of Pediatric Dermatology
(Weston, Lane, Morelli; Mosby, Inc.) which is used by clinicians worldwide and is
published in 4 languages.

He created the Genetic Skin Disorders clinic at the University of Colorado in 1998.

Copyright 2012 William L. Weston, M.D., All Rights Reserved