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Stevens-Johnson syndrome

Stevens-Johnson syndrome (SJS) is a severe and potentially life-threatening (15% of cases) disease, it is a hypersensitivity complex affecting the skin and the mucous membranes, a severe expression of erythema multiforme (EM) (and so SJS is also called erythema multiforme major). more...

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Symptoms

SJS is characterized by fever, sore throat, and headache leading to the sudden development of circular mucocutaneous lesions (target lesions) that can cover the majority of the skin. These lesions begin as macules and can develop into papules, vesicles, blisters, or urticarial plaques. The most extreme cases are termed Toxic Epidermal Necrolysis Syndrome (TENS) or Lyell's Syndrome, in these cases the entire skin is affected.

Treatment

Treatment is initially similar to that of patients with thermal burns, and continued care can only be supportive (e.g. IV fluids) and symptomatic (e.g. analgesic mouth rinse for mouth ulcer); there is no specific drug treatment (2002). An ophthalmologist should be consulted if eyes are involved. Treatment with corticosteroids is controversial since it might aggravate the condition.

Cause

The cause of SJS is either infections (usually following viral infections such as herpes simplex virus, influenza, mumps, cat-scratch fever, histoplasmosis, Epstein-Barr virus, or similar), drug-induced (valdecoxib, penicillins, barbiturate, sulfas, phenytoin, lamotrigine, nevirapine), malignancy-related (carcinomas and lymphomas), or idiopathic (50% of the time)

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Dangerous drug effect: protect yourself from a life-threatening reaction - Healthy family: news everyone need to stay well - Stevens-Johnson Syndrome
From Better Homes & Gardens, 4/1/03 by Linda Rao

What happens when the drug you're taking makes you feel worse than the sickness you're treating? Ask public relations consultant Valerie Chereskin, who, at age 35, suffered a severe drug reaction to an antibiotic she was taking for a sinus infection.

"At first, I had a slight fever and a burning sensation in my eyes," she recalls. "It felt like I was burning from the inside out." After two trips to the emergency room and several doctor visits, Valerie was finally diagnosed with Stevens-Johnson Syndrome (SJS), a potentially life-threatening drug reaction. Valerie spent two weeks in the hospital. By the time she was able to go home, she'd lost much of her hair, her eyelashes, eyebrows, fingernails, and toenails.

What is SJS? Although fully recovered now, Valerie's reaction was textbook SJS, which causes skin and mucous membranes--including those around your mouth, eyelids, and throat--to burn, blister, then peel. Considered relatively rare by medical experts, anecdotal evidence suggests SJS may be on the rise. And although it's more common in adults--40 percent of them women-children can also suffer from this condition.

What are the Culprits? "At least half of SJS cases are directly related to drug exposure," says Dr. Bernard Cohen, director of pediatric dermatology at the Johns Hopkins Children's Center in Baltimore. "Though any drug can cause reaction, anticonvulsants such as phenobarbital, as well as sulfa-based antibiotics and nonsteroidal anti-inflammatory drugs (NSAIDs) are often to blame."

Although only a few people in a million are diagnosed with SJS every year, the death rate from the syndrome can be as high as 20 percent, mostly due to secondary infections that take hold when so much skin is lost, says Dr. C. Stephen Foster, professor of ophthalmology at Harvard Medical School in Boston. The key to halting a severe reaction is to recognize the signs early on, Cohen adds.

What are the Treatments? Doctors first take people off the offending drug, then treat them with pain medication and intravenous fluids to prevent dehydration. Other treatments focus on limiting infection and other SJS complications.

COPYRIGHT 2003 Meredith Corporation

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