Stevens Johnson Syndrome, A Painful Skin Condition

Stevens Johnson Syndrome is named for Albert Mason Stevens and Frank Chambliss Johnson, American pediatricians who in 1922 jointly published a description of the disorder in the American Journal of Diseases of Children.

Stevens Johnson syndrome is a rare, serious disorder in which your skin and mucous membranes react severely to a medication or infection. Stevens Johnson syndrome presents a medical emergency that usually requires hospitalization. Treatment focuses on eliminating the underlying cause, controlling symptoms and minimizing complications.

Stevens Johnson Syndrome occurs twice as often in men as in women. Most cases of SJS appear in children and young adults under age 30.

According to the Mayo Clinic the signs and symptoms of Stevens Johnson syndrome include: facial swelling, tongue swelling, hives, skin pain, a red or purple skin rash that spreads within hours to days, blisters on your skin and mucous membranes, especially in your mouth, nose and eyes, and shedding (sloughing) of your skin.

SJS usually begins with flu-like symptoms such as fever, sore throat, cough, burning eyes, and fatigue, which is misdiagnosed and usually treated with antibiotics. It is followed by a painful red or purplish rash that spreads and blisters, eventually causing the top layer of your skin to die and shed. The ulcers and other lesions begin to appear in the mucous membranes, almost always in the mouth and lips but also in the genital and anal regions. Those in the mouth are usually extremely painful and reduce the patient’s ability to eat or drink. Conjunctivitis of the eyes occurs in about 30% of children who develop SJS. A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp.

Although Stevens Johnson Syndrome can be caused by viral infections, malignancies or severe allergic reactions to medication, the leading cause appears to be the use of antibiotics and sulfa drugs.

Stevens Johnson syndrome is a rare and unpredictable reaction. No test is available to help predict who is at risk. Some factors, however, may increase your risk of developing SJS, including: existing medical conditions, and genetics. Carrying a gene called HLA-B12 may make you more susceptible to Stevens Johnson Syndrome.

Medications are most often the cause of SJS. Drugs commonly associated with Stevens Johnson syndrome include: anti-gout medications, such as allopurinol, nonsteroidal anti-inflammatory drugs (NSAIDs), often used to treat pain, penicillins, which are used to treat infections, and anticonvulsants, which are used to treat seizures.
Infections can also cause Stevens Johnson syndrome. These infections include: Herpes (herpes simplex or herpes zoster), Influenza, HIV, Diphtheria, Typhoid, and Hepatitis. In some cases, SJS may be caused by physical stimuli, such as radiation therapy or ultraviolet light.

Recovery after Stevens Johnson syndrome can take weeks to months, depending on the severity of your condition. If your doctor determines that your case of Stevens Johnson syndrome was caused by medication, you’ll need to permanently avoid the medication and all others related to it.