What You Should Know About Stevens-Johnson Syndrome

Editor’s Note: Introducing Lisa Buben, guest contributor for Medtopicwriter. Please help us welcome Lisa to our guest contributor network for Medtopicwriter. A blogger and webmaster since 2009, Buben and her husband, Ken, own FancyScrubs, an online business that specializes in fluid repellent medical uniforms that protect healthcare workers from blood and bodily fluids. Check out these self cleaning uniforms and their popular nursing scrubs and clogs at FancyScrubs.com.

An allergic reaction to medication can cause Stevens-Johnson Syndrome. (photo credit: fotalia.com)

Most people had not heard of Stevens Johnson Syndrome until reading about Manute Bol, the famous basketball player in the 1980s, who died from complications of this disease. He was 7’7” and one of the tallest basketball players ever known.  On June 19, 2010, Bol died from acute kidney failure and complications from Stevens–Johnson syndrome at the University of Virginia Medical Center in Charlottesville, Virginia.

Researchers wanted to learn more about this rare disease and why physicians might not recognize the symptoms and miss the diagnosis. The first recorded case occurred in 1922.  The syndrome bears the names of Albert Mason Stevens and Frank Chambliss Johnson, American pediatricians, jointly published a description of the disorder in the American Journal of Diseases of Children in 1922. Physicians diagnose approximately 300 adults and children with the disease annually in the United States. Experts still do not know why more adults than children fall prey to Stevens Johnson Syndrome.

The skin and mucous membranes react severely to a medication or from an infection in this rare, but serious disorder. Often, Stevens-Johnson syndrome begins with flu-like symptoms, followed by a painful red or purplish rash that spreads and blisters, eventually causing the top layer of skin to die and shed. Mild forms of this disease can get better in 2-6 weeks. But, they can return and can prove difficult to treat, leading to more serious complications.

Signs and symptoms of Stevens-Johnson syndrome include:

  • Facial swelling
  • Tongue swelling
  • Hives
  • Skin pain
  • A red or purple skin rash that spreads within hours to days
  • Blisters on the skin and mucous membranes, especially in the mouth, nose and eyes
  • Shedding (sloughing) of the skin

If you have Stevens-Johnson syndrome, several days before the rash develops you may experience:

  • Burning Eyes
  • Fever
  • Cough
  • Sore Throat

When to See a Doctor

Stevens-Johnson Syndrome requires immediate medical attention. Seek emergency medical care if you experience any of the following signs or symptoms:

  • Unexplained, widespread skin pain
  • Facial swelling
  • Blisters on the skin and mucous membranes
  • Hives
  • Tongue swelling
  • A red or purple skin rash that spreads rapidly
  • Shedding of the skin

Complications

Possible complications of Stevens-Johnson syndrome include:

  • Secondary skin infection (cellulitis). This acute infection of the skin can lead to life-threatening complications, including meningitis — an infection of the membranes and fluid surrounding the brain and spinal cord — and sepsis.
  • Sepsis. Sepsis occurs when bacteria from a massive infection enter the bloodstream and spread throughout the body. Sepsis is a rapidly progressing, life-threatening condition that can cause shock and multiple organ failure.
  • Eye problems. The rash caused by Stevens-Johnson syndrome can lead to inflammation in the eyes. In mild cases, this may cause irritation and dry eyes. In severe cases, it can lead to extensive tissue damage and scarring within the eyes that can result in blindness.
  • Damage to internal organs. Stevens-Johnson syndrome can cause lesions on your internal organs, which can result in inflammation of your lungs (pneumonitis), heart (myocarditis), kidney (nephritis) and liver (hepatitis).
  • Permanent skin damage. When the skin grows back following Stevens-Johnson syndrome, it may have abnormal bumps and coloring (pigmentation). Visible scars may remain as well. Lasting skin problems may cause hair to fall out, and fingernails and toenails may not grow normally.
  • Death.

For further information, visit the Stevens Johnson Syndrome Foundation (Julie Foundation for Allergic Drug Reactions). The purpose of the foundation is to provide the public and medical communities with information on adverse allergic drug reactions. People report less than 1% of all adverse drug reactions to the FDA. The public needs to know what to look for when taking any drug and to seek medical attention immediately in the event of a reaction to prevent permanent damage or death. The SJS Foundation also conducts medical research about SJS/TEN.

The Stevens Johnson Syndrome Support Group originated in May of 1995. The Group’s purpose is to provide emotional support for people with Stevens Johnson Syndrome and Toxic Epidermal Necrolysis.

You can also read more about Julie’s story of recovery, an amazing story about a 10-month-old girl who suffered a Grand Mal seizure and the events that followed taking medication for the seizure. Julie’s story of recovery will inspire and awe anyone who reads about this courageous young girl and her family.

References

http://en.wikipedia.org/wiki/Manute_Bol

http://www.mayoclinic.com/health/stevens-johnson-syndrome/DS00940

http://www.mayoclinic.com/health/stevens-johnson-syndrome/DS00940/DSECTION=symptoms

Author: Lisa Buben, Fancy Scrubs

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