Stevens-Johnson Syndrome: Information on Causes, Diagnosis & Treatments

Rare Medication Reaction Stevens-Johnson syndrome (SJS) is a serious, but rare, disorder of the mucous membranes and skin. Typically, the syndrome is a reaction to an infection or certain medications. Many sufferers display painful red or purple rashes that will blister or spread. This is followed by the death of the affected skin outer layers before it sheds and finally heals. The effects of the syndrome are so critical that it requires emergency medical attention typically followed by hospitalization.

The emergency medical team will focus on eliminating the syndrome’s underlying cause and attempt to minimize complications and control the symptoms to allow the healthier skin to regrow. However, recovering from the syndrome often takes months, especially in severe cases. If Stevens-Johnson syndrome was caused by taking drugs, the patient must avoid the medication permanently and any other drug that contains the same active ingredients.

Who Is at Risk for Stevens-Johnson Syndrome?

Any individual can develop Stevens-Johnson syndrome. However, there are certain risk factors that increase the potential likelihood of developing the condition. These factors include:

  • Weakened Immune System – If the body’s immune system has been weakened due to an illness, infection or other condition, the patient has an increased potential risk of developing the syndrome. Weakened immune systems are often the result of an autoimmune disease, HIV, AIDS, or an organ transplant.

  • HIV Infection – The effects of an HIV infection are so severe that the incident rate of developing Stevens-Johnson syndrome is approximately 100 times higher compared to the general population.

  • Personal History – Anyone who has had a drug-induced four-month Stevens-Johnson syndrome is at risk of the syndrome recurring if the medication is taken again.

  • Family History – Patients with an immediate member of the family who has suffered from Stevens-Johnson syndrome or any condition related to toxic epidermal necrolysis is also highly susceptible to developing the syndrome.

  • Genetic Predisposition – Individuals who have acquired or developed the HLA-B 1502 gene are at great risk of developing Stevens-Johnson syndrome, especially when taking specific medications for treating seizures, mental illness or gout. This gene is most common in families of Indian, Southeast Asian, or Chinese descent.

Stevens-Johnson syndrome is often the result of an unpredictable reaction to a medication or infection where the cause is not easily identified. If the problem is drug-induced, common symptoms may not present themselves while the drug is being taken, or even up to two weeks once the medication has been stopped.

Complications

There are certain complications associated with acquiring Stevens-Johnson syndrome that include:

  • Sepsis (blood infection) – Bacterial infections that lead to sepsis in the bloodstream can spread throughout the body and progress rapidly causing organ failure, shock, or other life-threatening problem.

  • Cellulitis (secondary skin infection) – This type of infection can cause life-threatening complications like sepsis.

  • Lung Problems – Acquiring Stevens-Johnson syndrome could cause acute respiratory failure or another lung issue.

  • Eye Issues – Many patients suffering from Stevens-Johnson syndrome will develop a rash that causes the eyes to become inflamed. In mild cases, the patient will likely experience dry eyes or eye irritation. However, in severe cases, the patient experiences scarred tissue damage of the eye that leads to visual impairment and, in some cases, blindness.

  • Permanent Skin Damage – Stevens-Johnson syndrome almost always causes significant skin damage that through the healing process will become discolored or form abnormal bumps. Some experience extensive scars and long-lasting skin issues that could cause the loss of care or abnormal growth of toenails and fingernails.

Common Symptoms

The most common symptoms associated with Stevens-Johnson syndrome include:

  • Unexplained skin pain that spreads across a large area

  • Fever

  • Purple or red rashes skin that spread from the initial site

  • Blisters that appear on mucous membranes or skin around the eyes, nose, mouth, or genitals

  • Skin shedding that occurs days after the formation of blisters

  • A sore throat or mouth

  • Burning eyes

  • Coughing

  • Fatigue

A patient experiencing any of the symptoms above should seek immediate medical attention. Quick emergency medical treatment can minimize the severity of symptoms.

Diagnosing Stevens-Johnson Syndrome

Diagnosing Stevens-Johnson syndrome is straightforward. The doctor will take a complete medical history to list the drugs the patient has been taking, and determine if they are suffering or have suffered a major infection. Other procedures intestinal involve:

  • A Comprehensive Physical Examination – Stevens-Johnson syndrome can be identified through a comprehensive physical examination.

  • Skin Biopsy – A diagnosis of Stevens-Johnson syndrome is usually confirmed through a skin biopsy or a sample of skin is removed to be analyzed in a laboratory environment by a pathologist who views the sample under a microscope. In addition, this can rule out any other potential cause of a skin or membrane condition.

  • Cellular Culture – The doctor may take a sample of skin or oral swab to culture in a laboratory setting, allowing the sample to grow in a nurturing environment to make detection of the condition easier to analyze. The culture will also rule out or confirm any infection.

  • Imaging – If the doctor is unable to easily confirm a diagnosis of SJS, they may order an imaging x-ray test to rule out pneumonia or other chest-related problem.

  • Blood Testing – Taking and analyzing a sample of blood is a simple test for confirming the presence of an infection or rule out other causes.

Stevens-Johnson Syndrome Treatments

Developing Stevens-Johnson syndrome is an emergency situation tat requires hospitalization oftentimes, the patient will enter the burn unit or intensive care unit at the facility. The first step taken by the treating physician is to ensure that the patient discontinues any drug that might be the potential cause of the condition. However, it is often challenging to determine the exact cause, so the doctor will likely recommend the patient stop taking every nonessential drug.

The doctor will recommend two forms of treatment including medications and supportive care. In detail, these include:

  • Medications – There are specific drugs that are known to be highly effective at treating SJS. These include:
    • Painkillers to reduce many of the discomforting blisters and burns.

    • Antibiotics when required to cure or control infection

    • Drugs that induce the inflammation that occurs in mucous membranes and the eyes

    • Immune-related treatments including immune globulin and oral steroids used in severe cases

  • Supportive Care – While at the hospital being treated for Stevens-Johnson syndrome, the treating physician will order supportive care that includes:
    • Wound Care – The nursing staff will use wet, cool compresses to provide soothing to the blisters to allow them to heal. The team of medical professionals will gently remove dead skin at the affected area and cover the wound with creams or medicated dressing for protection.

    • Nutritional Supplementation and Fluid Replacement – Replenishing lost fluids from the body caused by the loss of skin is a crucial component of treatment to ensure the patient gets healthier. Usually, nutrients and fluids are delivered through a nasogastric tube inserted through the nose and into the stomach.

    • Eye Care – Likely the patient will be visited by an ophthalmologist (eye specialist) who will provide much-needed eye care if one or both eyes were damaged by the condition.

It is crucial to determine the underlying cause of SJS to ensure the reaction to the medication or infection has stopped. Full recovery often takes many months.

Sources:

http://www.mayoclinic.org/diseases-conditions/stevens-johnson-syndrome/home/ovc-20317097

http://www.mdedge.com/ccjm/article/91961/update-stevens-johnson-syndrome

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3863256/

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