Stevens-Johnson Syndrome (SJS) is a rare skin condition that was first discovered by American pediatricians in 1922. Albert Mason Stevens and Frank Chambliss Johnson reported two cases of young boys with “an extraordinary, generalized eruption with continued fever, inflamed buccal mucosa, and severe purulent conjunctivitis.” Previously, the 7 and 8-year-old boys were misdiagnosed with hemorrhagic measles. Even though this first case of SJS happened nearly 100 years ago, physicians today still misdiagnose the condition.

When SJS is present, patients experience skin cell death, resulting in the epidermis separating from the dermis. Often, this syndrome happens because of a “hypersensitivity complex” that affects the skin and mucous membranes. To this day, the exact biology of the condition is not fully understood. However, we do know that some of the most common causes of SJS are an allergic reaction to specific medications, such as antibiotics, anticonvulsants, and sulfa drugs.

Symptoms

Considered a type IV (subtype C) hypersensitivity reaction, SJS has long confused medical professionals and patients alike. Initial symptoms of the condition mimic those of the flu, making it difficult to diagnose. SJS may start with the following symptoms:

• Fever
• Body aches
• Widespread skin pain
• Red or purplish rash
• Cough
• Blisters
• Sores on the skin and the mucous membranes of the mouth, throat, eyes, and genitals
• Skin that peels off in sheets
• Swollen eyes
• Eyes sealed shut
• Pain with urination
• Drooling

If you have noticed any of the above symptoms, seek medical attention immediately. The sooner a doctor diagnoses SJS, the higher the chance of recovery. If left untreated, SJS may develop into Toxic Epidermal Necrolysis (TEN), a more severe version of the condition. In the past, medical experts thought SJS and TEN were different conditions. We now know they are of the same disease spectrum. SJS may affect up to 10 percent of the patient’s body surface, while patients with TEN experience skin detachment on 30 percent of the skin.

The purpose of today’s article is to take a look back at the history of SJS and what we’ve learned. By understanding the circumstances surrounding the first case of SJS back in 1922, we can see how the condition has progressed. Because SJS and TEN are so commonly misdiagnosed even today, some people may be able to file a medical malpractice lawsuit and recover damages. To learn more about filing a lawsuit following an SJS diagnosis, contact our McKinney Stevens-Johnson Syndrome lawyers.

History of SJS

As mentioned, we can trace the first case of SJS back to 1922. Albert Stevens was a prominent surgeon born in Rangoon, India, and Frank Johnson was an American pediatrician who graduated from Rutgers College in 1916. The two collaborated on a publication called “A New Eruptive Fever Associated with Stomatitis and Ophthalmia” in the American Journal of Disease of Children in December of 1922. The two young boys at the center of the first case had numerous skin eruptions that were dark red or purplish, as well as a fever, inflamed mucous membranes, and conjunctivitis. One of the boys had already lost his vision. Stevens and Johnson had never seen symptoms quite like this before, nor had anyone they consulted.

It is unknown when the condition was officially named Stevens-Johnson Syndrome, but the contribution these two professionals made to the medical community is invaluable. 

Drugs that cause an SJS interaction

During their studies, Stevens and Johnson found that the syndrome presented as an immune hypersensitivity that attacked the deepest layer of skin and mucous membranes. It appeared as if the skin was burning from the inside out, which is why SJS and TEN cases today are often treated at local burn units.

SJS is still a mystery in many ways. But we have learned a lot since the first case emerged. For one, doctors now believe an allergic reaction to some medications leads to SJS, including:

• Allopurinol
• Advil/ibuprofen
• Ansaid/Flurbiprofen
• Celebrex/Celoxicab
• Children’s Advil/Motrin
• Mood stabilizers like Tegretol (Carbamazepine)
• Lamictal/Lamotrigine
• Nonsteroidal anti-inflammatory drugs
• Sulfa antibiotics
• Dilantin/Phenytoin
• Indocin
• Lodine/Etodolac
• Motrin
• Tolectin/Tolmetin
• Vioxx/Rofecoxib

If you have taken a prescription or over-the-counter drug that caused a severe reaction, call your physician immediately. The sooner you diagnose SJS, the better.

Risk Factors of SJS

Many of the drugs linked to SJS are very common and taken by people on a regular basis. SJS is a rare skin condition, meaning not every person who is prescribed the above drugs will develop the disease. There are certain risk factors for SJS, including:

• Viral infections (herpes, hepatitis, viral pneumonia, and HIV)
• A weakened immune system (due to certain autoimmune conditions like lupus, HIV and AIDS, chemotherapy, and organ transports, for example)
• A history of Stevens-Johnson Syndrome
• A family history of Stevens-Johnson Syndrome

Finding Relief from SJS 

Once diagnosed, numerous steps can provide patients with relief during treatment at the hospital or burn unit. These treatments may include:

• Pain killers to address widespread skin discomfort
• Cold compresses against the skin
• Application of a plain, unscented moisturizer
• Replacement fluids
• Antiseptic mouthwashes
• Topical corticosteroids to control skin inflammation
• Antibiotics (in some cases)
• Eye drops or eye ointments

Please keep in mind these treatments should only occur under the direct supervision of a specialist.

SJS Today

Sadly, many doctors and other medical professionals still do not know the why behind Stevens-Johnson Syndrome. Some believe it is the result of damage to the blood vessels in the skin, but this is just one theory. Technologies will continue to evolve and give us a greater understanding of rare conditions like SJS and TEN moving forward. If you or a loved one has been diagnosed with SJS, please contact the Stevens-Johnson Syndrome lawyers at Wormington & Bollinger. Depending on the circumstances surrounding your case, you may be able to file a lawsuit and seek damages for medical malpractice. To schedule a free consultation to discuss your case, give us a call today.