Steven - Johnson Syndrome

1 What is Steven - Johnson Syndrome?

Steven-Johnson syndrome (SJS) is a deadly skin disease that is usually due to a drug reaction. Several drugs that have been linked to SJS include  non-steroidal anti-inflammatory drugs (NSAIDS), Allopurinal, henytoin and some types of anticonvulsants. 

This condition can also be caused by bacterial infections. Although it occurs mostly in older people, it can also affect any other age group. Women are more often affected compared to men. 

People living with AIDS are at an increased risk of contracting Steven-Johnson Syndrome and thus they are urged to be vigilant about the signs of this disease.

2 Symptoms

In its early stages, Steven-Johnson Syndrome usually has non-specific symtoms such as headaches, coughs, body aches ,malaise and fever. These symptoms may be followed u by a rash that starts on the face but later it spreads to the whole body. 

Sometimes the rash can form blisters which can be mostly seen in the area of the eyes, mouth and vagina. The mucous membranes can be inflammed. Later on ulcers can develop in the mucous membranes mostly in the mouth and lips , in the genital and anal areas. Conjunctivitis occurs mostly in children who have SJS.

3 Causes

Medications have been shown to cause Steven - Johnson Syndrome. It can happen due to the adverse effects of some antibiotics, alloopurinol,anticonsulsive drugs and NSAIDS. 

The second most common cause of SJS ,especially in children is infection. This includes upper respiratory infections and pharyngitis. 

Viruses that can cause SJS include: Epstein-Bar virus and influenza virus. Bacterial infections include bete-hemolytic streptococci and diphtheria. This disease has has genetic predisposition.

4 Making a Diagnosis

In order for Steven - Johnson Syndrome to be diagnosed,certain tests have to be done. These include:

  • a physical exam-in which the diagnosis of SJS is made based on medical history,
  • physical exam and signs and symtoms which the patient presents with. 

A skin test is also done to confirm the diagnosis. This involves taking a skin samle (biopsy) and studying it under the microscope to find abnormal changes in the skin.

5 Treatment

Knowing the cause of Steven-Johnson Syndrome can be a very crutial part in determining the method of treatment. For example, if the cause is a certain drug withdrawing the drug can help to eleviate the symptoms. 

Antibiotics can also be given if the cause is a bacterial infection. At first treatment is similar to that of thermal burns i.e. administering intravenous fluids or parenteral feeding and symptomatic treatment. Other supportive measures include the use of  topical pain anaesthetics and antiseptics. 

Beyound supportive treatment no other treatment is accepted. The use of corticosteroids in SJS, however this has been a matter of controversy. Other agents used include cyclophosphamide and cyclosporin.

6 Prevention

Avoiding certain drugs is one way to prevent Steven - Johnson syndrome especially in people who are predisposed.

For examle, Asians are sensitive to carbamazepine-an anti-epileptic drug, so avoiding this drug can be prevent a person from having SJS.

7 Risks and Complications

There are several complications associated with Steven - Johnson syndrome (SJS).

SJS has a mortality rate of around 5% depending on the surface area of the body that is affected. Organ failure, cornea scratching and blindness are the ossible outcomes although they do not occur in every case. 

Other complications are: secondary skin infections like cellulitis ,sepsis and permnent skin damage. Problems with skin make the haie to fall out or abnormal growth of nails.