Stevens-Johnson syndrome: causes, diagnosis and treatment

Stevens-Johnson Syndrome (SJS) is a rare and extremely serious condition that causes the skin to loosen and peel off.

It was formerly known as Lyell’s syndrome, and it is sometimes incorrectly called “Steven Johnson syndrome”. SJS is related to toxic epidermal necrolysis (TEN), and they are similar diagnoses, but TEN is a more severe state.

In addition to 80 percent of cases, SJS is caused by an unpredictable adverse reaction to medication. It is less often caused by infections.

Researchers report that there is four to five cases of SJS per 1 million adults per year.

Although SJS is rare, it is also life-threatening and must be treated immediately.

MEDICAL EMERGENCY

If you think you have SJS, call emergency services or go to an urgent care facility right away. SJS often progresses along the following path:

  • You have taken any new medications in the past 8 weeks.
  • You have flu symptoms.
  • You have a rash on your face or chest that spreads to mucous membranes, which extend from your nose to your lungs.
  • You feel blisters.

The hallmark symptom of SJS is a rash with blisters that involves your mucous membranes.

In many cases, you will experience other symptoms 1 to 3 days before the onset of the eruption. Often the first symptom is fever. Other early symptoms may include:

When the rash begins, your skin may have darker pink patches in the middle. These areas can be flat or slightly raised. The spots may take on a darker red or purple color as symptoms progress.

The rash usually starts on the face and chest before spreading to mucous membranes, most of the time your lips and the inside of your mouth, but it could also appear on your:

  • eyes and eyelids
  • genitals
  • esophagus
  • upper respiratory tract
  • anus

In some places, the rash blisters and the epidermis – the outermost layer of your skin – dies and peels off.

SJS rashes can occur anywhere, but they usually start on your face or chest and then spread throughout your body. The mucous membranes are also affected, with about half of people with SJS developing rashes at three separate mucosal sites.

Below are pictures of typical rashes caused by SJS.

SJS is most often caused by an immune response to certain medications. It is also possible for certain infections to cause SJS.

Some experts have suggested that a combination of medications and infections together could cause SJS, while in some cases the trigger is never identified.

Medications

While over 100 drugs have been associated with SJS, they generally fall into a handful of categories. SJS develops within a few days to 8 weeks after first taking the new drug.

Medications associated with SJS include:

  • anticonvulsants, such as lamotrigine, carbamazepine, phenytoin, and phenobarbital
  • antibiotics, such as penicillins, cephalosporins, quinolones, and minocycline
  • nonsteroidal anti-inflammatory drugs (NSAIDs) of the oxicam class
  • acetaminophen
  • sulfa drugs, including cotrimoxazole and sulfasalazine
  • allopurinol, which is sometimes used to treat gout, kidney stones, and other conditions
  • nevirapine, which is used to treat HIV
  • contrast agents used in some medical tests

infections

There are two main categories of infections associated with SJS: viral and bacterial. Viral infections associated with SJS include:

Bacterial infections associated with SJS include:

Vaccines

There have been reports of SJS linked to vaccinations, although it is extremely rare, totaling about a dozen known cases associated with almost as many different vaccines. There is no clear pattern or specific vaccine to know.

SJS can affect anyone, but it is more common in women and the elderly. It has also been reported that SJS is more common in children.

Certain medical conditions are risk factors for SJS, including:

Your genes are also a risk factor for SJS. If a close family member has had SJS or TEN, your risk of SJS is increased.

Gene variations HLA-B are associated with a increased risk of SJS and can sometimes be found in people of various ethnic identities, including:

  • Han Chinese
  • Thai
  • malaysian
  • South India
  • Taiwanese
  • Japanese
  • Korean
  • European

Without genetic testing, you probably won’t know if your genes are affected. Doctors may order a genetic test to assess your level of risk before treating you with certain medications.

If you have had SJS or TEN before, you are at higher risk for another event and should avoid known triggers.

SJS and TEN are on the same disease spectrum, but they are different diagnoses. The determining factor is the amount of skin affected:

  • 10% or less is considered SJS.
  • 10-30% is considered overlap, abbreviated as SJS/TEN.
  • 30 percent or more is considered TEN.

Although SJS is the least serious of these diagnoses, it is still life-threatening and must be treated immediately.

Because SJS affects your skin and mucous membranes, these areas are at risk for complications, which can include:

You may also experience serious SJS complications inside your body, including:

If you are diagnosed with SJS, you will be immediately admitted to a hospital, potentially to an intensive care unit (ICU), specialist burns unit, or dermatology unit.

If you are taking medications that could cause SJS, you will stop taking them. Treatment will focus on managing symptoms and preventing complications, such as secondary infections.

Skin care will be one of the main goals of treatment, including cleansing, dressing, and removal of dead tissue.

Other treatments include:

SJS should be treated immediately.

Although the first symptoms are usually flu-like, you should seek immediate medical attention if you also meet any of the following criteria:

  • started taking new medications in the past 8 weeks
  • have a rash that you think is SJS
  • have a family history of SJS or TEN
  • have a previous diagnosis of SJS or TEN

There are no specific test or criteria diagnose SJS.

A doctor, often a specialist like a dermatologist, will diagnose your condition based on your medical history and the symptoms you experience.

Doctors may take a skin sample, called a biopsy, or perform other diagnostic tests to help confirm SJS and rule out other conditions.

The only method of preventing SJS is to minimize your risk as much as possible.

If you have ever had SJS or TEN, avoid triggers and medications associated with SJS. This is also relevant if you have not had SJS or TEN but a family member does.

Before you begin taking medications that may trigger SJS, your doctor may examine your genes for variations that may increase your risk of SJS.

Recovery from SJS will be different for everyone, depending on the severity of symptoms and complications. SJS is sometimes deadly – apparently 2 to 5 percent cases – but these numbers have improved as treatments improve.

The skin will begin to regrow in 2 to 3 weeks but could take several months to fully recover. Other symptoms or complications will have a range of recovery times and may have permanent effects.

SJS can take a mental toll in addition to the physical symptoms. If you’ve had SJS, talk to a healthcare professional if you feel depressed or anxious.

SJS is a rare disease, but when it happens, it’s a medical emergency.

Symptoms may look like the flu at first, but a rash usually appears within a few days. The rash will spread to the mucous membranes and peel off and blister.

Certain medications can cause SJS. Less commonly, it can be the result of an infection. Your genes may increase your risk of SJS.

If you are diagnosed with SJS, you can expect to be admitted to hospital immediately. The sooner you start treatment, the better.

After you have had SJS, your doctor will advise you on how to prevent it from happening again.