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Quick reference medical handouts used by Pediatric offices


What is Stevens-Johnson Syndrome


Our skin is the body's largest organ and as such is affected by many diseases. A child's skin can tell both parents and doctors lot about the youngster's overall health. The nails, teeth, hair, lips, the lining inside the mouth and over the eyes can provide a unique window to what might be going on inside the body. From vitamin deficiencies to autoimmune diseases, a number of illnesses can manifest themselves first in the skin.

One such disease, Stevens-Johnson syndrome, is an extreme allergic reaction, usually to a drug, but also to certain bacterial and viral infections. The top three drugs associated with Stevens-Johnson syndrome and commonly used in treating kids are:

  • Carbamazepine (Tegratol - a medication used to treat seizures
  • Cotrimoxazole (Septra®, Bactrim® and a host of other generic trimethoprim-sulfazoxazole preparations)These are sulfa antibiotic used in curing urinary tract infections and in the treatment/prevention of ear infections
  • Sulfadoxine and pyrimethamine - used in the treatment of malaria and in children with immunodeficiency diseases

Stevens-Johnson syndrome seems to be more common in boys and has a tendency to occur in early spring and winter.

Typically, the illness begins within the first two weeks of taking the drug (if that is the cause). Most parents think that their child as the flu, since the youngster has nonspecific upper respiratory tract infection symptoms. Fever, sore throat, chills, headache, and achiness may be present. Abruptly skin lesions appear. They appear as wide spread skin eruptions consisting of large dark-red spots (caused by microscopic hemorrhage into the skin - purpura) with overlying blisters which then rupture leaving necrotic (dead tissue) centers. The typical lesion has the appearance of a target and does not usually itch the child. Infection may then set it causing skin scarring and the need for additional medication.

Lesions may continue to erupt in crops for as long as 2-3 weeks. But it is the presence of lesions on the membranes lining the eyes, mouth , and other organ systems that make Stevens-Johnson syndrome so dangerous. Involvement of the lining membranes includes redness, swelling , sloughing, blistering, ulceration, and tissue death.

Eye involvement can include ulcerations of the cornea, anterior uveitis, visual impairment and blindness . The eyes will get puffy, swollen, and gritty. There may be so many lesions inside the mouth that the child is unable to eat or drink with cracking or small blisters on the lips and mouth. Esophageal narrowing may occur when there is extensive involvement of the esophagus. If the urinary tract becomes involved, the child may complain of pain while urinating and have difficulty in voiding.

Medications Implicated in Causing
Stevens-Johnson Syndrome

Antibiotics

Sulfa
Penicillins
Gentamycin
Ciprofloxacin

Tetracycline

Nonsteroidal Anti-
inflammatory Drugs

Ibuprofen
Oxyphenbutazone
Naproxen
Indomethacin

Seizure medications

Phenytoin Sodium
Carbamazepine
Phenobarbital

Diuretics

Furosemide


Since Stevens-Johnson Syndrome is potentially fatal (death can occur in up to 15% of untreated cases ), hospitalization of the child is required, often in the intensive care unit. Treatment includes removing the offending medication and meticulous care of the skin. Because the skin has extensive blistering and ulceration, treatment is similar to that of a severe burn. In addition, treatment consists of maintaining fluids and electrolytes, caring for of any mouth or eye lesions and providing proper nutritional support. Steroids may provide benefit to the child, especially when given during the first week of the illness. Unfortunately, there is no treatment that will stop Stevens-Johnson Syndrome once it starts. All that can be done for the child is to treat the many manifestations of the illness.

During the course of the disease, children should be monitored for signs of secondary infections, dehydration, electrolyte imbalances, and malnutrition. The disease is generally self-limited and usually resolves in 4 to 6 weeks.

What lessons can be learned from illnesses like Stevens-Johnson syndrome ? With bad drug reactions being the fourth leading cause of death in the United States, it is important that all parents think carefully before giving medications to their children and do so only when absolutely necessary. The overuse of antibiotics in particular has not only produced a new generation of bacteria who are resistant to antibiotics, but their inappropriate use puts the child at risk for developing potentially fatal diseases like Stevens-Johnson syndrome.

For more information, contact the Stevens Johnson Syndrome Foundation at 9285 Utica St., Westminster, CO., 80030, (303) 430-9559; (303) 487-9359 (Fax). UPDATED 01-07-09

 

 

 

As a reminder, this information should not be relied on as medical advice and is not intended to replace the advice of your child’s pediatrician. Please read our full disclaimer.

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