Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

This dangerous condition can be a reaction to drugs like Lamictal

Stevens-Johnson Syndrome - one form of rash
Stevens-Johnson Syndrome - one form of rash. Erythema Multiforme Pictures.com

Stevens-Johnson syndrome is a serious disease that can affect your skin, eyes, and mucous membranes, causing a fever and a severe skin rash that blisters and peels. It can be disfiguring and even life-threatening, especially if it's not treated immediately. This condition can appear as a severe reaction to certain medications, including the anticonvulsant medication ​Lamictal (lamotrigine), which is used to treat epilepsy and as a mood stabilizer in the treatment of bipolar disorder. A more severe form of the disease is called toxic epidermal necrolysis.

A Disease Spectrum

While both Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are on the spectrum of this life-threatening skin disease, SJS is on the less severe side of the spectrum and TEN is on the severe end. Both are potentially fatal. Many medical professionals refer to The factor that distinguishes the two conditions from each other is the percentage of body surface that has peeling skin. This is defined as:

  • SJS: Under 10 percent of the body has skin that's peeling
  • TEN: Over 30 percent of the body has skin that's peeling
  • SJS/TEN overlap: Between 10 and 29 percent of the body has peeling skin

Blisters in the mucous membranes, usually the nose, eyes, mouth, and genitals, also occur in more than 90 percent of people with any of these three conditions.

For the purposes of this article, the term "SJS/TEN" will be used to include all three of the reactions described above.

Causes

SJS/TEN is most often caused by a reaction to a certain medication or by an infection, but in over one-third of cases, the exact cause can't be found. For some people, there may be a genetic component.

Medications

Medications that have been associated with SJS/TEN include:

  • Anticonvulsants, especially Tegretol (carbamazepine), phenobarbital, Lamictal (lamotrigine), and Dilantin (phenytoin)
  • Aloprim and Zyloprim (allopurinol), which treat gout and kidney stones
  • Sulfonamide antibiotics like Bactrim or Septra (sulfamethoxazole-trimethoprim), Azulfidine (sulfasalazine), Gantrisin (sulfisoxazole), and penicillin
  • Viramune (nevirapine) for treating human immunodeficiency virus (HIV) infection
  • Pain relievers like Motrin or Advil (ibuprofen), Tylenol (acetaminophen), and Aleve (naproxen)

The risk of developing SJS/TEN occurs within the first eight weeks of treatment with an associated medication, usually within four days to four weeks after you first start taking it continuously. Although some skin reactions may occur with Lamictal, most of these will not go on to a serious rash or Stevens-Johnson syndrome.

Infections

Infections that can lead to SJS/TEN include:

  • Pneumonia, particularly when it's caused by the Mycoplasma pneumoniae virus
  • Herpes simplex or herpes zoster
  • HIV
  • Hepatitis A

Genetics

There are genetic variations that have been pinpointed as increasing the risk of developing SJS/TEN in response to a trigger like medication. These genetic changes are mostly found in genes that play a role in the immune system, especially the HLA-B gene, a part of the human lymphocyte antigen (HLA) complex. Despite these gene variations, most people who have them never develop SJS/TEN, even when exposed to the medications that can cause it. This likely means that there are a variety of factors that come into play when someone develops SJS/TEN and genetics only plays one of several roles.

Early Symptoms

If you're taking Lamictal or any other medication that's associated with SJS/TEN, it's important for you to be aware of the symptoms so you can recognize them when they first occur. You should bring any rash to the attention of your doctor.

You may have flu-like symptoms one to three days before the rash appears such as:

  • Headache
  • Fever
  • Sore throat and/or mouth
  • Cough
  • Burning or itchy eyes
  • Fatigue
  • Body aches
  • Joint pain
  • Diarrhea
  • Nausea
  • Vomiting

If these symptoms occur, there's no particular reason to be worried—it could be something else entirely. However, if the more serious skin reactions appear later, be sure to tell your doctor about the earlier symptoms you had as well.

Later Symptoms

Serious SJS/TEN symptoms that occur as the disease progresses include:

  • A purple or red rash that starts on your face, neck, and chest and quickly spreads
  • Widespread pain in your skin
  • Swelling of your face and/or tongue
  • Blisters on your mucous membranes, especially in your mouth, nose, or eyes, but possibly in the genital area too
  • Hives
  • Shedding or peeling of skin
  • Chills
  • Hair loss

If you have these symptoms, stop taking Lamictal or the triggering medication at once and seek immediate medical attention. This condition is an emergency and you need to get treated as soon as possible.

Complications

Complications that can result from SJS/TEN include:

  • Bacterial infections, including sepsis, a life-threatening infection in your bloodstream that spreads to your whole body quickly and can cause multiple organ failure and death
  • Pneumonia, which may lead to acute respiratory failure
  • Permanent scarring, discoloration, or bumps in your skin when it grows back
  • Eye issues like dryness, pain, and scarring of your corneas, which can cause impaired vision

Diagnosis

When you go in for a diagnosis, your doctor will do a physical exam and possibly a skin biopsy. If your doctor thinks you might have an infection, you may have a chest X-ray to check for pneumonia, blood cultures to look for signs of infection, and/or a skin or mouth culture to rule out or identify infection. Typically, diagnosis is fairly simple and is based on your symptoms, how much of your skin is involved, the way your affected skin looks, and how quickly the rash is spreading.

Treatment

If you're diagnosed with SJS/TEN, you'll need to be hospitalized in a burn unit or the intensive care unit (ICU), if possible. Treatment for this condition is similar to that of burn victims and may include supportive care measures such as:

  • Immediate discontinuation of any medication(s) that may have caused SJS/TEN
  • Caring for your blisters and sores with measures like cool compresses, medicated ointments, and bandages
  • Keeping you hydrated with intravenous (IV) fluids and salts, since the skin loss from the blisters causes you to lose fluid
  • Feeding you through a tube that goes through your nose into your stomach if you can't eat normally
  • Medications to help control any pain you might experience
  • Eye care from an ophthalmologist, a doctor specially trained in treating eyes, if needed
  • Antibiotics if you develop an infection

Other medications and treatments that may be used depending on your individual situation may include:

  • Systemic corticosteroids
  • IV immune globulin
  • Sandimmune (cyclosporine)
  • Tumor necrosis factor inhibitors like Remicade (infliximab)
  • Plasmapheresis, a procedure in which the liquid in your blood, the plasma, is separated from the blood cells to remove the antibodies or drugs that might be causing SJS/TEN

Research on these treatments is conflicted and limited, but many treatment centers do use them.

Outlook

Recovery can take weeks or months, depending on how severe your case is. Around 25 percent of people who develop SJS/TEN die overall, which includes about 10 percent of SJS patients and 30 percent of TEN patients. In children, the death rate is less than 10 percent.

For those people who recover, it's necessary to avoid any drug(s) that caused SJS/TEN because you can get develop it again when you're reexposed to the drug. You'll also need to stay away from drugs that are chemically related to the medication(s) that triggered SJS/TEN.

There are other long-term issues you should look for as well regarding your skin, eyes, mouth, lungs, and mental health.

Skin

As mentioned above, you may have permanent skin discoloration, bumps, or scarring when your skin grows back. Your hair and/or nails may also fall out, but they will grow back.

Eyes

Somewhere between 50 percent and 90 percent of people have continued eye problems like dryness, ingrown eyelashes, sensitivity to light, and keratitis. You may have visual impairment from scarring in your corneas, or, rarely, blindness. The chance of developing eye disorders in the future, even years later, is higher, so be sure to see an ophthalmologist if you develop any strange eye symptoms.

Mouth

You may also have mouth issues that stem from your bout of SJS/TEN like inflammation, discomfort, dry mouth, tooth decay, or gum infections. Kids who have had SJS/TEN may have problems with the way their teeth grow and/or the roots in their teeth.

Lungs

Having had SJS/TEN can lead to chronic bronchitis or chronic bronchiolitis with obstructive changes, bronchiectasis, and other obstructive disorders.

Mental Health

Mental health disorders like anxiety and depression can occur after having SJS/TEN. You may also notice a difference in your quality of life in terms of physical health. If you think you have symptoms of any mental health disorder, be sure to talk to your doctor. Treatment can help immensely.

Sources:

Genetics Home Reference. Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis. National Institutes of Health. U.S. National Library of Medicine. U.S. Department of Health and Human Services. Updated September 18, 2018. https://ghr.nlm.nih.gov/condition/stevens-johnson-syndrome-toxic-epidermal-necrolysis

Gonzalez ME. Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN). Merck Manual: Consumer Version. Updated March 2018. https://www.merckmanuals.com/home/skin-disorders/hypersensitivity-and-inflammatory-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

High WA, Roujeau J. Stevens-Johnson . UpToDate. Updated May 25, 2018. https://www.uptodate.com/contents/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-management-prognosis-and-long-term-sequelaeand Toxic Epidermal Necrolysis: Management, Prognosis, and Long-Term SequelaeSyndrome

High WA, Roujeau J. Stevens-Johnson . UpToDate. Updated August 16, 2018. https://www.uptodate.com/contents/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-pathogenesis-clinical-manifestations-and-diagnosisand Toxic Epidermal Necrolysis: Pathogenesis, Clinical Manifestations, and DiagnosisSyndrome

Mayo Clinic Staff. Stevens-Johnson Syndrome. Mayo Clinic. Updated March 9, 2018. https://www.mayoclinic.org/diseases-conditions/stevens-johnson-syndrome/symptoms-causes/syc-20355936

Was this page helpful?