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.
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.
SJS mostly occurs in toddlers and in young children.
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.
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.
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.
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.
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.
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.
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.
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