Stevens-Johnson syndrome

RARE BUT POTENTIALLY fatal, Stevens-Johnson syndrome (SJS) is an immune-complex-mediated hypersensitivity reaction. It's characterized by high fever and rash. Bacterial and viral infections, drugs, and malignancies can all trigger SJS. Erythema multiforme minor is a milder form of SJS and toxic epidermal necrolysis syndrome (TENS) is a more severe form.

Initially, SJS causes high fever, cough, sore throat, and burning eyes. Within 3 days, red or purplish lesions appear on the face, torso, and mucous membranes. These lesions may be flat or raised and have overlying blisters or necrotic centers.

Mucosal inflammation is extremely painful and may include erythema, edema, sloughing, blistering, ulceration, and necrosis. The oropharynx, eyes, genitalia, and anus are typically affected.

Complications of severe SJS or TENS may include massive edema, fluid loss, electrolyte imbalance, pneumonia, esophageal strictures, nephritis, anemia, lymphopenia, and neutropenia.

In its early stage, the disorder is easily mistaken for influenza or chicken pox. It's diagnosed on the basis of clinical signs and symptoms and the patient's medical and drug history. Direct immunofluorescence studies and skin biopsies can confirm the diagnosis.

Many drugs can trigger SJS, including anticonvulsants (such as carbamazepine), sulfa drugs (such as cotrimoxazole), and nonsteroidal anti-inflammatory drugs (such as ibuprofen). Identify any drugs that the patient started taking up to 3 weeks before the onset of SJS.

Patients at risk for developing SJS include those undergoing multiple-- drug therapy or bone marrow transplantation and those with systemic lupus erythematosus or HIV infection.

What's the treatment and prognosis?

If a drug has triggered SJS, stop giving it. Otherwise, care is mainly supportive and based on the patient's condition. Treatment is generally similar to that provided for bum patients, including guarding against dehydration and malnutrition. Administering steroids is controversial.

As many as 15% of patients with severe SJS die, usually from sepsis or pulmonary complications. The lesions of surviving patients heal in about 4 weeks. Patients may have mucosal scarring, and function may be impaired in involved organ system.

BY SUE M. PARINI, RN, CIC, BS. MA Manager, Infection Control * Paradise Valley Hospital National City, Calif.

Copyright Springhouse Corporation Apr 2001
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