Stevens-Johnson Syndrome and Its Causes, Symptoms, and Triggers

Stevens-Johnson syndrome (SJS) is a rare condition of the skin and mucous membranes. It is usually thought of as a severe reaction to a medication or infection, though some experts think it is a separate condition.

There is also a severe form of Stevens-Johnson syndrome called toxic epidermal necrolysis (TEN) or Lyell's Syndrome.

This article discusses Stevens-Johnson syndrome and its causes. It also goes over the symptoms and triggers of this condition.

What Is Stevens-Johnson Syndrome?

Stevens-Johnson syndrome can be life-threatening and can cause serious symptoms, such as large skin blisters and shedding of a child's skin.

Children of any age and adults can be affected by Stevens-Johnson syndrome, although people who are immunocompromised, such as those with HIV, are likely more at risk.

Stevens-Johnson syndrome is usually thought of as a severe form of erythema multiforme, which is itself a type of hypersensitivity reaction to a medication or infection. This can occur with prescription medications and with (including over-the-counter drugs) or infections like herpes or walking pneumonia caused by Mycoplasma pneumoniae.

Other experts think Stevens-Johnson syndrome is a separate condition from erythema multiforme. These experts acknowledge two forms of erythema multiforme: erythema multiforme minor and erythema multiforme major (Stevens-Johnson syndrome).

Symptoms of Stevens-Johnson Syndrome

Stevens-Johnson syndrome generally starts with flu-like symptoms, such as a fever, sore throat, and cough. Next, within a few days, a child with Stevens-Johnson syndrome will develop:

• A burning sensation on the lips, inside of their cheeks (buccal mucosa), and eyes
• A flat red rash, which may have dark centers, or develop into blisters
• Swelling of the face, eyelids, and/or tongue
• Red, bloodshot eyes
• Sensitivity to light (photophobia)
• Painful ulcers or erosions in the mouth, nose, eyes, and genital mucosa, which can lead to crusting

Complications of Stevens-Johnson syndrome can include corneal ulceration and blindness, pneumonitis, myocarditis, hepatitis, hematuria, kidney failure, and sepsis.

A positive Nikolsky's sign, in which the top layers of a child's skin comes off when rubbed, is a sign of severe Stevens-Johnson syndrome or a sign that it has evolved into toxic epidermal necrolysis.

A child is also classified as having toxic epidermal necrolysis if they have more than 30% epidermal (skin) detachment.

What Is Most Likely to Cause Stevens-Johnson Syndrome?

Although more than 200 medications can trigger Stevens-Johnson syndrome, the most common include:

• Anticonvulsants (epilepsy or seizure treatments), including Tegretol (Carbamazepine), Dilantin (Phenytoin), Phenobarbital, Depakote (Valproic Acid), and Lamictal (Lamotrigine)
• Sulfonamide antibiotics, such as Bactrim (Trimethoprim/Sulfamethoxazole), which is often used to treat UTIs and MRSA
• Beta-lactam antibiotics, including penicillins and cephalosporins
• Nonsteroidal anti-inflammatory drugs, especially of the oxicam type, such as Feldene (Piroxicam) (not usually prescribed to children)
• Zyloprim (allopurinol), which is typically used to treat gout

Stevens-Johnson syndrome is usually thought to be caused by drug reactions, but infections can also trigger it, including those caused by:

• Herpes simplex virus
• Mycoplasma pneumoniae bacteria (walking pneumonia)
• Hepatitis C
• Histoplasma capsulatum fungus (Histoplasmosis)
• Epstein-Barr virus (mono)
• Adenovirus

Cam Stevens-Johnson Syndrome Be Cured?

The treatments for Stevens-Johnson syndrome typically begin by stopping whatever drug may have triggered the reaction. The patient will also receive supportive care until they recover, which usually takes about four weeks. These patients often need to be placed in an intensive care unit. Treatments might include:

• IV fluids
• Nutritional supplements
• Antibiotics to treat secondary infections
• Pain medications
• Wound care
• Steroids and intravenous immunoglobulin (IVIG), although their use is still controversial

Stevens-Johnson syndrome treatments are often coordinated in a team approach, with the ICU doctor, a dermatologist, an ophthalmologist, a pulmonologist, and a gastroenterologist.

Parents should seek medical attention immediately if they think their child might have Stevens-Johnson syndrome.

Summary

Stevens-Johnson syndrome is a severe reaction to a medication or antibiotic that affects the skin and mucous membranes. It causes serious symptoms like large blisters and skin loss. Stevens-Johnson syndrome can be triggered by medications, including certain antibiotics, anticonvulsants, and prescription NSAIDs. Infections with certain viruses or bacteria can also trigger it.

Stevens-Johnson syndrome is usually treated in an intensive care unit with IV fluids, pain medication, wound care, and other supportive treatments.