Stevens-Johnson syndrome

Stevens-Johnson syndrome is a serious disorder of the mucous membranes. It is thought to be an allergic reaction. The exact cause of Stevens-Johnson syndrome can't always be identified. But known causes include:

• Reaction to certain medications, including sulfonamide antibiotics, penicillin and phenytoin (Dilantin)
• Cocaine use
• Complication of viral or bacterial infection, such as herpes, influenza, AIDS, diphtheria, typhoid and hepatitis
• Radiation therapy for cancer

Stevens-Johnson syndrome has signs and symptoms similar to those of erythema multiforme, a condition causing painful blistering and erosion of the skin and mucous membranes. However, in Stevens-Johnson syndrome, the blistering and systemic symptoms are more severe and extensive — often involving the mouth, nose, eyes, vagina, urethra, lower respiratory tract and digestive tract.

Stevens-Johnson syndrome occurs most often in children and young adults. Signs and symptoms often appear suddenly and include:

• Flu-like signs and symptoms, such as aches, coughing, headaches and fever
• Red rash that spreads
• Blistering of the skin and mucous membranes

A doctor may diagnose Stevens-Johnson syndrome based on:

• Medical history
• Signs and symptoms
• Physical examination of the affected skin or mucous membranes
• Removal of a small piece of affected tissue (biopsy) for examination under a microscope

Treatment of Stevens-Johnson syndrome is directed at the underlying cause, when possible, and at managing the signs and symptoms. Treatment may include:

• Pain relievers
• Topical anesthetics
• Corticosteroids or other immune system modulating medications
• Antibiotics, if infection develops
• Fluid and nutritional supplementation, when needed

Recovery may take several weeks. When Stevens-Johnson syndrome is diagnosed within 24 to 48 hours of onset, prompt treatment may shorten the duration of the illness. Up to 15 percent of cases of Stevens-Johnson syndrome result in death.