Stevens-Johnson Syndrome

Stevens-Johnson syndrome (SJS) is a rare, serious skin and mucous membrane disorder. SJS can involve the oral, nasal, eye, vaginal, urethral, gastrointestinal, and lower respiratory tract mucous membranes. This disorder eventually causes the top layer of skin to die and shed. This is an emergency medical condition usually needing hospitalization. The more skin that is lost, the more serious and life-threatening the disorder becomes.

CAUSES

SJS is usually caused by an allergic reaction to medicine. Medicines such as antibiotics (sulfa, penicillin) and antiseizure medicines (phenytoin) have been prescribed to more than ⅔ of all patients with SJS. Other medicines known to cause SJS include medicine used for gout (allopurinol), cocaine, other antiseizure medicines (carbamazepine, barbiturates), and nonsteroidal anti-inflammatory drugs (NSAIDS, such as ibuprofen and naproxen). Other causes of SJS are viral infections (mycoplasma), bacterial infections, and rarely cancer. Sometimes the cause is unknown.

SYMPTOMS

SJS often begins with several days of flu-like symptoms, such as:

  • Fever.

  • Sore throat.

  • Cough.

  • Burning eyes.

After the first several days, your mucous membranes become inflamed. A painful red or purplish rash develops on the face and trunk, spreads, and creates blisters over your skin. Other common signs of SJS include:

  • Swelling in the face.

  • Hives.

  • Skin pain.

  • Joint aches.

  • Sensitivity to light (photophobia).

  • Difficulty eating or swallowing due to pain in the mouth and throat.

  • Difficulty urinating.

  • Low blood pressure and fast heart rate.

  • Nosebleeds and sore mucous membranes.

  • Blisters on your skin and mucous membranes, especially in your mouth, nose, and eyes.

  • Shedding of your skin.

  • Tongue swelling.

DIAGNOSIS

  • Your caregiver can often recognize SJS from your medical history, a physical exam, and your symptoms.

  • To confirm the diagnosis, your caregiver may take a tissue sample (biopsy) from your skin to be examined under a microscope.

  • Blood tests and other general lab work may also be done.

TREATMENT

Treatment focuses on getting rid of the cause, if possible, and minimizing or preventing complications. If the cause of SJS can be removed and the skin reaction can be stopped, your skin may begin to grow again within several days. In severe cases, full recovery may take weeks to months. Prompt treatment usually shortens the length of the illness.

  • SJS requires hospitalization, often in an intensive care unit or burn unit.

  • Any medicines that could be causing SJS are stopped.

  • Pain medicines will be given as needed for discomfort.

  • Antibiotics are given for infection, if needed.

  • Immunoglobulin may be given by vein to try to stop the disease process.

  • Skin loss can result in large losses of fluid from your body. These fluids must be replaced. You may receive fluids and nutrients through a tube placed through your nose and into your stomach (nasogastric tube).

  • Blisters that have not broken may be left to heal on their own. Your caregiver may gently remove dead skin and put a bandage (dressing) on those areas.

  • Skin grafting may be used on large areas, but this is rarely needed.

PREVENTION

Preventing a recurrence of SJS is critical. A recurrence is usually more severe than the first episode and can be life-threatening.

  • If your SJS was caused by a medicine, be sure to avoid that medicine and others in the same drug class. This information will be listed in your medical chart.

  • If herpes virus caused your reaction, you may need to take daily antiviral medicines to prevent a recurrence.

HOME CARE INSTRUCTIONS

  • Know what caused your reaction. If a medicine caused your reaction, learn the name of that medicine and any other related medicines that could cause a recurrence.

  • Let all your caregivers know about your history of SJS. If the reaction was caused by a medicine, provide your caregivers with the name of that medicine.

  • Wear a medical bracelet or necklace that says you had SJS and what caused it.

SEEK IMMEDIATE MEDICAL CARE IF:

  • You cough up thick saliva mixed with mucus (sputum).

  • You have a headache.

  • You feel very tired.

  • You have joint aches and pain.