Stevens-Johnson Syndrome (SJS)

Stevens-Johnson Syndrome (SJS)

Stevens-Johnson Syndrome or SJS is a rare but severe adverse skin reaction that typically emerges in response to medication. SJS is classified as an Adverse Drug Reaction or ADR.
It is characterized by fever, flu-like symptoms, and painful red or purple rashes on the skin. This condition typically requires hospitalization and careful medical care, making it extremely important for patients to report symptoms
to their doctor as soon as possible after discovery.

What are the Causes of Stevens-Johnson Syndrome (SJS)?

Not all causes of SJS are known. Sometimes, the condition can manifest itself due to an infection unrelated to ADR. Patients with certain conditions—such as HIV/AIDS and certain types of pneumonia—are at heightened risk of developing Stevens-Johnson Syndrome (SJS).
In most cases, SJS occurs as a negative reaction to medication. There is no one single medication that causes this condition. In fact, virtually any medication—including over-the- counter medications—can cause ADR conditions such as SJS. Certain drugs are more commonly linked to SJS than others, such as anti-convulsant medications (Carbamazepine and Lamotrigine) and antibiotics like penicillin. However, in most cases, the reaction has more to do with individual biology than it does with the drug.
Stevens-Johnson Syndrome (SJS) tends to be more common in children than in adults.

What are Symptoms and Risks of Stevens-Johnson Syndrome (SJS)?

Some of the symptoms of SJS can mimic other conditions. Early on, the condition can be characterized by fever and other flu-like symptoms, which can make it easy to mistake SJS for something else. Over time, SJS becomes more apparent, manifesting as blisters or rashes all over the skin. SJS affects the body’s mucous membranes, with patients frequently complaining of blisters or swelling in the mouth, eyes, nose, ears, or around the genitals.

Patients who report SJS to their eye doctors often complain of conjunctivitis or pink eye. Pink eye is characterized by the inflammation of the inner eyelid and the eye’s outer membrane.
After the first few days of blisters and rashes, the skin starts to peel. SJS patients shed the skin in affected areas, leaving behind raw, painful, and infection-prone wounds all over their bodies. This causes SJS to have a high-risk nature, as it can lead to infection of the secondary skin and blood. These infections are life-threatening and can ultimately escalate to shock and organ failure. If Stevens-Johnson Syndrome (SJS) progresses, it can lead to permanent tissue damage of the skin and eyes. As skin grows back following SJS, it may have bumps, unusual coloring, or visible scars. In severe SJS cases, tissue damage can extend to the eyes and cause long-term visual impairment.

How to Treat Stevens-Johnson Syndrome (SJS)?

The first step to SJS treatment is diagnosis because it is vital to rule out other conditions that could be causing similar signs and symptoms. If your doctor determines you have SJS, he or she will ask you to discontinue use of any non-essential medications. If you are taking multiple medications, it can be difficult to isolate which one might be causing an ADR. As such, it is easier to cease all medications to stop complications.
Patients are often cared for by burn unit teams since SJS rashes and skin peeling symptoms strongly resemble severe burns. Due to the way the condition affects the body, treatment will usually involve consultations with experts in ophthalmology, dermatology and infectious diseases. Treatment itself includes skin care to sooth and disinfect wounds, eye care to prevent permanent tissue damage, and bodily fluid replacement.

Scleral lenses are among the most common eye treatments for SJS. A scleral lens is a contact lens that sits on the sclera, or the white part of the eye. These lenses have a wider diameter and more surface area than the average contact lens. As such, they are ideal for protecting the cornea. They form a fluid- filled reservoir over the cornea that is extremely beneficial for patients suffering from dry eyes.
For SJS patients, scleral lenses have several advantages. Eye dryness is one symptom of SJS, which can make it painful or uncomfortable to blink. Scleral lenses help reduce irritation or pain and protect the cornea. Even if SJS patients aren’t dealing with dry eyes, scleral lenses can provide overnight protection that reduces corneal scarring and helps the eye heal.

Scleral lenses can help improve the vision through a cornea scarred from Stevens-Johnson Syndrome (SJS).