Stevens Johnson syndrome (SJS) and Toxic Epidermal Necrolysis Syndrome (TENS) another form of SJS is a rare but serious disorder of the skin and mucous membranes. It usually comes about as a result of a reaction to a medication or an infection. Adverse drug reactions (ADRs) account for approximately 150000 deaths per year in the U.S. alone, making drug reactions the fourth leading cause of death in the U.S. There is very limited data on ADRs in Africa. SJS is one of the most debilitating ADR recognized. It was first discovered in 1922 by pediatricians A.M. Stevens and S.C. Johnson after diagnosing a child with severe ocular and oral involvement to a drug reaction. ‘So If your name na Steven or Johnson abeg no vex ooh’
What can cause SJS?
SJS is a rare and unpredictable reaction. The doctor may not be able to identify the exact cause, but usually the condition can be triggered by any medication or an infection. The most commonly implicated drugs are; anti-gout medications such as allopurinol, pain relievers like paracetamol and ibuprofen, medications to fight infections such as penicillins and sulpha drugs, medications to treat seizures or mental illness and radiation therapy. Infectious causes include Herpes, Pneumonia, HIV and Hepatitis.
Who can get SJS and TENS?
Although SJS afflicts people of all ages, a large amount of victims are children. More female cases have been reported than male, however it does not discriminate against anyone. There are various factors that increase your risk of developing SJS such as viral infections (e.g. HIV or Hepatitis), weakened immune system (such as in HIV/AIDS or organ transplant), previous history of SJS, family history of SJS, having a certain gene (If you have a gene called HLA-B1502 you have increased risk of SJS. Chinese, Southeast Asians and Indians are more likely to carry this gene. Note that SJS is not contagious and we therefore don’t need to be scared about infecting others or getting infected.
SJS: Know the Signs and Symptoms
Recognition of the early symptoms of SJS and prompt medical attention is the most invaluable tool in minimizing the possible long-term effects SJS may have on its victims. Symptoms include;
· Facial swelling, tongue swelling
· Skin pain, and a red or purple rash that spreads within hours to days.
· Blisters in mouth, ears, eyes, nose and genital area,
· Swelling of eyelids, red eyes.
· If you have SJS several days before the rash develops you may experience flu-like symptoms. IF YOU NOTICE TWO OR MORE OF THESE SYMPTOMS, CONTACT YOUR DOCTOR IMMEDIATELY
SJS and TENS are life-threatening reactions. If left untreated, they can result in death. Complications include permanent blindness, asthma, secondary skin infection, blood infection, damage of internal organs and permanent skin damage.
The first most important step in management is to discontinue medications that may be causing SJS immediately to prevent complications. Treatment for SJS is good supportive care. Because patients literally burn from the inside out, burn, infectious disease, ophthalmology and dermatology teams are recommended. IV fluids and high calorie formulas are given to promote healing. Antibiotics are given when necessary to prevent secondary infections such as sepsis. Pain medications such as morphine are administered to make the patient as comfortable as possible. Most SJS patients can be managed in medical intensive care unit (ICU) or pediatric ICU. Patients with TENS are treated in a burn unit. If the underlying cause of SJS can be eliminated and the skin reaction stopped, new skin may begin to grow over the affected area within several days to months depending on how severe. If you have SJS be sure to know what caused your reaction. Always inform your healthcare providers (doctor, pharmacist, nurse…) if you suspect any reactions to a drug for professional advice.