What Is Steven Johnson Syndrome?

Steven Johnson syndrome (SJS) is a rare and severe condition that affects a person's skin and mucus membranes and can cause a person to loose around 10 % of the outer skin layer. It is estimated that around 90% cases of SJS are misdiagnosed as other skin conditions, such as chicken pox. This should not deter you from obtaining a diagnosis. Besides, as SJS is classed as a medical emergency, so immediate medical attention and hospitalization are required.

Symptoms of Steven Johnson Syndrome

The symptoms related to SJS are similar to other skin conditions; hence there are consistent errors in diagnosis. Those with SJS may experience the following symptoms:

  • Swelling of the face
  • Swelling of the tongue
  • Hives
  • Skin pain
  •  fast spreading skin rash of a red or purple color
  • Blistering of the skin and mucus membranes (mouth, nose, eyes and genitals)
  • Skin shedding
  • Stevens Johnson syndrome can also cause fever, fatigue, cough, sore throat and mouth, and burning eyes. These symptoms can develop days before any appearance of a rash.

Some symptoms are similar to that of common cold, so if you experience any of the former mentioned symptoms, it is essential that you visit your doctor immediately and find out if it is SJS.

What Causes Steven Johnson Syndrome?

It can often be hard for your health care professional to efficiently determine the cause of SJS. The common theory is that it is often a result of an infection, or a reaction to medication. Infections such as herpes, hepatitis, pneumonia and HIV have all been known to cause Steven Johnson syndrome. Medications pain relievers, penicillin, anti-gout medication and radiation therapy also have been known to cause SJS.

Risk Factors of Steven Johnson Syndrome

As mentioned, SJS is a serious condition that requires immediate medical attention. There are numerous risk factors that increase your susceptibility to the condition, some of which are listed below:

  • Viral infections increase your risk of developing SJS.
  • People with weakened immune systems are at an increased risk of SJS.
  • A history of SJS: If you have previously had medication-induced SJS and you take the medication again, you may have a relapse.
  • A family history of SJS: If a member of your close family has had SJS, this may increase your risk of developing the condition. Specifically, a gene, called HLA-B 1502 is thought to increase the risk of developing SJS.

How to Treat Steven Johnson Syndrome

Once it has been determined that you have SJS, immediate treatments are needed to subdue the condition. Below are some of the treatment methods currently used by health care professionals:

1. Stop Nonessential Medications

As Steven Johnson syndrome can be caused by medication and it is difficult for your doctor to determine what medication is causing the condition. So you may be first recommended by your doctor to discontinue the consumption of all nonessential medication.

2. Supportive Care

  • Get enough fluid and nutrition: Your body requires nutrients to repair and rebuild, and it is essential to remain hydrated. In most cases, you will first be attached to an IV, providing you with the fluid and nutrition needed, and then be bed fed via a tube running through your nose and into your stomach.
  • Wound care: Whilst in the hospital, the staff will ensure that your skin remains clean, and may bandage severely affected areas. Cool, wet compression may also be applied to help soothe the blisters as they heal.
  • Eye care: This may be provided by an eye specialist (ophthalmologist), and can include using specialized eye drops and creams to prevent the area from drying out.
  • TPN is a type of liquid nutrition that gives your body all the essential nutrients required to function efficiently. TPN is often used when a person has problems eating or digesting solid or liquid foods. It is inserted in your vein with a catheter or needle for about 10-12 hours, 5 times per week so as to provide vitamin, sugar, mineral and protein to your body.

3. Take Medications

Some of the medications currently used by health care providers to treat Steven Johnson syndrome are listed below:

  • Antibiotics may be offered to treat and prevent infection.
  • Antacids will likely be needed if sores develop in your stomach, causing bleeding.
  • Blood thinners may be applied to prevent any blood from clotting.
  • Eye drops help prevent dryness of eyes, as well as reduce the risk of infection.
  • Immune globulins help increase the strength of your immune system and may be offered to treat or prevent infection.
  • Pressors will often be offered if your blood pressure is low. Pressors will increase your blood pressure and return it to a normal level, which helps in protecting the brain, heart, kidneys, lungs and other organs.

4. Surgery

If your skin does not heal properly, then surgery may be required. Dirt or dead tissue may be removed via debridement and a skin graph may be performed to help repair and cover up areas that lost skin. 

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