Stevens-Johnson Syndrome

Stevens-Johnson Syndrome It is a severe and rare condition wherein the mucus membranes and the skin have a serious reaction to infection or a certain drug. On most occasions, the disorder starts with flu-like signs which are then followed by the appearance of a painful, purplish or red rash that spreads and causes blisters. This will eventually result in the uppermost layer of the skin to die and fall off. It is considered as a medical emergency that generally involves hospitalization. The aim of the treatment is to eradicate the existing cause, control the symptoms and minimize the complications.

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The recovery period of Stevens-Johnson syndrome can last from a few weeks to many months and usually depends on the severity of the condition. In case, a certain drug was diagnosed as the underlying cause of the condition, then the affected individual has to stop the intake of that medication and others like it.

 Symptoms

Some of the symptoms of Stevens-Johnson syndrome are listed below:

  • Swelling of the face
  • Hives
  • Swelling of the tongue
  • The skin may be painful
  • The skin and mucous membranes, particularly the nose, mouth and eyes may experience blisters
  • A purplish or reddish rash that tends to spread within a few hours or days
  • The skin may slough off or shed

Individuals affected by Stevens-Johnson syndrome may experience the below mentioned symptoms, many days prior to the appearance of the rash:

  • The eyes may experience burning sensations
  • Increased soreness of the throat
  • Cough
  • Fever

Some of the possible complications that may result due to Stevens-Johnson syndrome are discussed below:

  • The acute infection that affects the skin may result in secondary skin infections such as cellulitis, meningitis, etc. which can be life threatening. Meningitis is an infection of the membranes and fluid that surround the spinal cord and the brain.
  • The rash that results due to Stevens-Johnson syndrome can lead to many eye problems like eye inflammation. Mild cases of the condition may lead to dry eyes and irritation. Severe cases of the disorder can result in extreme tissue damage as well as scarring inside the eyes which can eventually lead to blindness
  • One of the worst complications of Stevens-Johnson syndrome is sepsis wherein bacteria tends to enter the body via an infection site and spreads to all the parts of the body through the bloodstream. Sepsis tends to spread rapidly and can result in organ failure, shock and even death.
  • There may be permanent damage of the skin. Once Stevens-Johnson syndrome is treated and the skin grows back, it may develop pigmentation and irregular bumps. There may be scarring of the skin. The hair may not grow back or fall out. Also, the toenails and fingernails may grow abnormally
  • Stevens-Johnson syndrome can also result in damage of the internal organs as well as formation of lesions in them. This can lead to inflammation of the heart, liver, lungs and kidneys.

Causes

The definite cause of Stevens-Johnson syndrome cannot be always determined. In most cases, the disorder is caused due to an allergic reaction to an infection, a medication or an illness.

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Medications are the primary cause of Stevens-Johnson syndrome. Some of drugs that are known to cause the condition are as follows:

  • Allopurinol and other anti-gout drugs
  • Different types of penicillin that are consumed to cure infections
  • Nonsteroidal anti-inflammatory drugs or NSAIDs that are used to alleviate pain
  • Anticonvulsants that are used to manage seizure disorders

Some infectious causes of Stevens-Johnson syndrome include the following:

  • Herpes zoster or Herpes simplex
  • Typhoid
  • HIV
  • Influenza
  • Diphtheria
  • Hepatitis

In certain cases, the condition may be caused as a response or reaction to physical stimuli such as ultraviolet light or radiation therapy

Some of the risk factors that increased the vulnerability to developing Stevens-Johnson syndrome include the following:

  • Individuals who have a gene known as HLA-B12 are at greater risk to the condition
  • Presence of conditions such as lupus, HIV and other disorders that impair the immune system

 Treatment

The treatment of Stevens-Johnson syndrome involves hospitalization of the affected individual, often in the burn unit or the ICU

  • The first step of the treatment is to stop the intake of all medications. It is not possible to identify the exact medication that has caused the condition, hence the consumption of all medications.

While at the hospital, the Stevens-Johnson syndrome patient may get the following supportive care:

  • The use of wet, cool compresses to soothe the wounds while they heal
  • Removal of the dead skin and dressing the wounds with a topical anesthetic
  • Stevens-Johnson syndrome can result in sever loss of fluids. Hence the fluid content of the body is replaced via manual, medical means
  • An eye doctor has to be consulted to prevent any damage to the eyes
  • The doctor may use antihistamines to ease itching, topical steroids to alleviate inflammation of the skin, pain killer medications and antibiotics for the control of infection.
  • Skin grafting may be required, if large sections of the body are affected by Stevens-Johnson syndrome.
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