Definition

Stevens-Johnson Syndrome (SJS), previously known as Lyell's syndrome, is a rare and potentially fatal skin and mucous membrane reaction. Toxic epidermal necrolysis (TEN) is a more severe form of this condition.

Stevens-Johnson syndrome (SJS) is a rare but serious condition that affects about 2 to 7 out of every million people each year. It is more common than Toxic Epidermal Necrolysis (TEN). In over 80% of the cases, medications are the most common cause of SJS. Symptoms of the syndrome may start with flu-like complaints, followed by a painful rash and skin peeling. Usually, the skin reactions only affect less than 10% of the body's surface area. However, in some cases, SJS and TEN overlap with skin surface damage on 10-30% of the body surface area.

Stevens-Johnson syndrome is an emergency condition that requires immediate medical attention.

Causes

Stevens-Johnson Syndrome (SJS) can have various causes, but often it is a reaction to medications, infections, or both. In fact, medications are responsible for over 80% of SJS cases. Drug-induced reactions can occur within a few days to eight weeks after starting the medication, and even up to two weeks after stopping the drug. In some cases, the exact cause of SJS cannot be identified.

Medications that can cause SJS include:

• Uric acid-lowering drugs, such as allopurinol, especially at doses greater than 100 mg per day
• Anti-seizure drugs, such as lamotrigine, carbamazepine, phenytoin, phenobarbitone
• Drugs for mental disorders, such as anti-seizure drugs and antipsychotics
• Sulfonamide compounds, such as cotrimoxazole, sulfasalazine
• Antibiotics, such as penicillins, cephalosporins, quinolones, and minocycline
• Antiviral nevirapine
• Pain medications, such as acetaminophen, ibuprofen, and naproxen sodium
• Contrast agents for radiology examinations

Meanwhile, infections such as pneumonia (lung infection) and HIV can also cause SJS.

Risk factor

SJS can occur in anyone but is more common in older people and women. In general, factors that can increase a person's risk of developing SJS are:

• HIV infection, the incidence of SJS in people with HIV is 100 times higher than in people without HIV
• People with weakened immune systems such as people who have undergone organ transplants, people with HIV/AIDS, and people with autoimmune diseases
• Cancer patients, especially blood cancers, have a higher risk of developing SJS
• Previous history of SJS, if you have had SJS due to medication, then you are at risk of developing SJS again if you take the same medication
• Family history of SJS, if you have a sibling with SJS, then you are at a higher risk of developing the same condition
• Genetic factors, some people have genetic variations that make them more susceptible to developing SJS, especially if the person is also taking medications such as anticonvulsants, gout medications, or other medications for mental disorders

Symptoms

SJS usually begins with flu-like symptoms after exposure to a specific cause or trigger. These symptoms occur one to three days before the rash appears and include:

• Fever
• No appetite
• Cough and runny nose
• Sore mouth and throat
• Fatigue
• Hot or burning sensation in the eyes
• Muscle pain

Along with the progression of the disease, other symptoms will appear namely:

• Unexplained painful sensations on the skin in all parts of the body
• A painful reddish or purplish-colored rash that spreads all over the body
• Blisters on the skin and mucous membranes of the mouth, nose, eyes, and genitals. A few days after the blisters form, the top layer of the affected skin will die, peel off, and begin to heal within a few days

Diagnosis

Your doctor can usually identify the presence of SJS based on your medical history, history of medications you are currently taking or have recently stopped, and by performing a physical examination. The diagnosis of SJS is generally apparent from the appearance of the skin reaction on the patient's body. To confirm the diagnosis and rule out other possible causes, the doctor may perform a biopsy examination by taking a skin sample for examination under a microscope.

In addition, to look for infection, your doctor may take samples from your skin, tissues, or body fluids for laboratory culture. Depending on your symptoms, your doctor may also perform radiology tests, such as a chest X-ray, to look for pneumonia. Blood tests may also be used to confirm infection or other causes other than SJS.

Management

Treatment for SJS focuses on eliminating the cause, treating the wound, controlling pain, and minimizing complications as the skin heals. Treatment for SJS usually requires an intensive care unit or burn treatment room.

The first and most important thing in treating SJS is to stop any medications that may be the cause. If two or more drugs are currently being consumed, your doctor may recommend stopping all non-essential drugs.

After that, the therapies that will be given in the hospital are:

1. Fluid replacement therapy and nutrition

Fluid replacement is an important part of treatment, as significant fluid loss can occur. Fluids and nutrients are administered through a tube inserted through the nose into the stomach (nasogastric tube).

2. Wound care

Cool, wet compresses can help provide comfort while the blisters heal. The doctor may also gently remove dead skin and apply petroleum jelly (Vaseline) or a medicated wound dressing to the skin wound.

3. Eye care

An ophthalmologist may also be involved in the eye care.

Medications used to treat SJS include:

• Medications to control pain
• Medications to reduce inflammation of the eyes and mucous membranes (steroid ointments or drops)
• Antibiotics to control infection if needed
• Oral or injectable medications, such as corticosteroids and injectable immunoglobulins

If the cause of SJS is ruled out or eliminated and the skin reaction stops, new skin will begin to grow within a few days. In severe cases, complete recovery may take several months.

Complications

SJS can cause some complications such as:

• Skin sloughing due to SJS can cause the body to lose fluids. In addition, wounds to the mouth and throat also make it difficult to eat and drink, resulting in dehydration
• Blood infection (sepsis). Sepsis occurs when bacteria from an infection enter the bloodstream and spread throughout the body. Sepsis is a rapidly deteriorating and life-threatening condition that can lead to shock and organ failure
• Eye disorders. The rash caused by SJS can lead to eye inflammation, dry eyes, and sensitivity to light. In rare cases, SJS can cause visual impairment and even blindness (rare)
• Pulmonary disorders. SJS can cause an emergency situation where the lungs cannot deliver enough oxygen to the blood
• Permanent skin damage. When the skin grows back after SJS, the skin growth may be imperfect. The skin may be bumpy and darker in color, and may also be accompanied by scars. This skin damage can lead to hair loss and impaired growth of fingernails and toenails

Prevention

SJS can be caused by both medications and infections. If the cause is a drug, then prevention is to avoid that drug or similar ones for life. This is very important as recurrences are usually more severe than the first episode and can be fatal.

If you have a family member who has suffered from SJS after taking certain medicines, you should also avoid those medications as this condition sometimes runs in families.

When to see a doctor?

This condition is a medical emergency and requires immediate medical attention. If you experience any signs and symptoms of SJS, seek medical attention immediately.

Looking for more information about other diseases? Click here!