This is a quite rare health condition that negatively affects the skin and mucous membranes. It is known as Stevens-Johnson Syndrome (SJS) and it commonly begins with flu-like symptoms. The primary cause of this condition is a reaction to specific medication. A late symptom of this disease includes a painful rash that spreads in different body parts. Thereafter, the top of the skin dies and the healing begins within a few days.
In most cases, SJS is a medical emergency and needs hospitalization. The treatment goal involves determining and removing the cause and reducing the symptoms. Commonly, it takes from weeks to months to fully recover.
In case you ignore this disease it may lead to a more severe form known as toxic epidermal necrolysis (TEN). It impacts roughly 30% of the skin’s surface. Furthermore, if you experience SJS due to a medicine, you should avoid it permanently.
Symptoms
The symptoms can change as the disease progresses. Check below some early symptoms:
However, when SJS develops, you may also experience:
- Shedding of the skin
- Skin blisters
- Red or purple rash
- Unusual widespread skin pain
Immediately contact a healthcare provider or call 911 if you experience any of the previous symptoms.
Causes
Diagnosing this condition is difficult sometimes. In other words, doctors cannot determine what triggers this disease. Mostly, it occurs due to medicines, infections, or both. Check below some medicines that can provoke SJS:
- Anti-gout medications (such as Allopurinol)
- Drugs used in the seizure treatment (including Anticonvulsants and Antipsychotics)
- Antibacterial sulfonamides (such as Sulfasalazine)
- Nevirapine
- Pain relievers (such as Ibuprofen, Naproxen, and Acetaminophen)
The most common infections that cause SJS are HIV and pneumonia (severe respiratory infection).
Risk Factors
Anyone can develop this disease and the following factors could increase your risk of developing SJS. Examples include:
- HIV infection – If you experience this condition are more likely to get SJS.
- Weakened body’s defense (immune system) – People with weakened immune systems are more likely to develop SJS and other diseases. The most common factors that contribute to a weakened body’s defense include autoimmune conditions, HIV/AIDS, organ transplants, and cancer medications.
- Cancer – Those who suffer from cancer (especially blood cancer) are at elevated risk of SJS.
- Medical history of SJS – If the disease is triggered by a medicine and you experience a recurrent disease, you may need to use this medication again. This can lead to SJS.
- Family history – If a family member has this condition, you are at higher risk of developing it.
- Genetic factors – Some people develop SJS due to having certain genes along with taking medicines for gout, mental disorders, or seizures.
Complications
- Dehydration – This occurs in the places where the skin is shed. Dehydration also can happen due to sores in the mouth or throat which make difficult fluid intake.
- Blood infection (sepsis) – When the bacteria that cause infection enters the bloodstream it provokes sepsis. This is a progressive condition that can lead to shock, organ failure, or even death.
- Eye disorders – SJS may provoke inflammation or dry eye, and sensitivity to light. If you experience a severe form of SJS visual impairment or even blindness may occur.
- Lung problems – Acute respiratory failure can happen if the lungs do not enter enough oxygen.
- Permanent skin damage – Some people after recovery may notice that the skin has certain bumps or unusual color (dyspigmentation).
Is it Possible to Prevent Stevens-Johnson Syndrome?
- Genetic tests before using medicines – These tests can help you determine if you have specific genes that may provoke SJS before treatment.
- Do not use medicines that triggered SJS before – After recovery, it is advised to avoid trigger medications and other similar drugs. Moreover, the recurrent SJS will be more severe than the first episode and sometimes may be fatal.
Diagnosis
The following tests and procedures are usually performed by doctors to diagnose Stevens-Johnson syndrome. Examples include:
- Doctors will ask you questions about your medical history and perform a physical examination.
- Skin biopsy – This test is used to exclude other causes and confirm the disease.
- Culture – A sample of the skin, fluid, or tissue is collected by the doctor and sent to a laboratory for testing (culture). This test can help to exclude an infection.
- Imaging – This test is usually used to determine whether you have pneumonia or not. However, it depends on the symptoms you have.
- Blood tests – These tests help your physician to identify the cause of SJS or an infection.
Treatment
People with SJS usually need hospitalization and sometimes intensive care.
The treatment usually begins with interrupting the medications that potentially provoke this disease. For those who administer more medications at the same time, it can be challenging to determine which one causes SJS. That’s why it is advised to stop taking all medicines.
Medicines
Check below the most common drugs prescribed by the doctors:
- Painkiller medicine that helps to lessen discomfort
- Medicines used to decrease inflammation of the mucous membranes and eyes
- Antibiotics (these medications are used to control infection)
- Physicians may also prescribe the following medicines for the treatment of SJS. These include corticosteroids, IV immune globulin, and others. As per studies, Etanercept and Cyclosporine are effective in the SJS treatment.
Usually, the new skin starts growing within a few days if the underlying cause of SJS is stopped. However, it may last several months for the skin to grow in severe cases.
Supportive Care
This supportive care is received by patients in the hospital usually. For example:
- Fluid replacement and nutrition – An important part of the SJS treatment is fluid replacement because due to skin loss, a person may experience significant fluid loss. Doctors usually use a tube (nasogastric tube) to give you fluids and nutrients.
- Wound care – Physicians will remove the dead skin and will apply medicines to the affected area.
- Eye care – Sometimes, people with SJS may require care from an ophthalmologist (eye specialist).
Frequently Asked Questions
What are the possible SJS complications?
People who suffer from this syndrome may experience some complications including skin problems. For example skin infections, skin color changes, and scarring. However, some organ problems also can occur. This condition often provokes problems in the liver, lungs, kidneys, and eyes.
What is the life expectancy for Stevens-Johnson syndrome?
Some people may experience serious complications including pneumonia, sepsis, multiple organ failure, and others. Approximately 10% of people with SJS die from this condition. Moreover, this disease is fatal up to 50% in those who suffer from toxic epidermal necrolysis. Talk with your doctor for more details.
What are the medicines that usually provoke SJS?
- Anticonvulsants (such as Lamotrigine, Carbamazepine, Phenytoin, and Phenobarbitone)
- Allopurinol (especially if you are taking it in doses more than 100 mg daily)
- Sulfonamides (such as Cotrimoxazole and Sulfasalazine)
If you have additional questions, ask your healthcare professional.
