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What is Steven Johnson Syndrome ?

Steven Johnson Syndrome  is caused by an allergic reaction to some medications. The syndrome causes fever and flu-like symptoms and those affected suffer great pain and discomfort. Skin rashes and blisters also appear in different parts of the body. Children are more prone to be affected by the syndrome.

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Steven Johnson Syndrome requires immediate treatment to prevent it from causing more serious health problems which could result to death. Treatment for the condition is focused towards addressing the specific symptoms experienced by the child. Parents should be vigilant about giving over-the-counter medications to their children as most of these drugs do not have forewarnings about serious side effects.

Steven Johnson Syndrome facts

Steven Johnson Syndrome or SJS is medically referred to as an immune complex mediated hypersensitivity condition wherein the patient suffers from a potentially deadly allergic reaction. In SJS, the mucous membranes and skin become inflamed.  The mucous membranes can be found on the lungs, the respiratory system as well as the digestive system, thus, allowing the syndrome to affect various parts of the body. SJS causes symptoms similar to erythema multiforme (EM) and toxic epidermal necrolysis (TEN). But SJS is more serious than EM because it damages the mucous membranes and endangers major organs. On the other hand, SJS is less serious than TEN which causes large blisters and swamping of the mucous membranes and skin.

Statistics about Steven Johnson Syndrome

It is estimated that around 1 to 6 out of 1 million individuals are affected by SJS every year. It affects not only children and adolescents but even very young babies as well. It had been noted that a number of cases of SJS in children involve boys, and usually happen during springtime and wintertime. Around 5% of the treated cases of SJS result in death and roughly 15% for untreated SJS.

Steven Johnson Syndrome in Children and causes

SJS in children generally occur after an infection in the upper respiratory system. Typically, parents administer over the counter medications to treat the associated symptoms of upper respiratory infection. However, they don’t realize that these drugs can cause an allergic reaction that could potentially kill the child. The mucous membranes get inflamed and this inflammation may spread to other parts of the body, especially to the vital organs. Delayed treatment will eventually result in lesions on the skin and organs, hindering their normal functioning which would ultimately lead to death.

Some of the medications that could cause Steven Johnson syndrome are:

  • Antibiotics

These are used in treating infections and examples of these are penicillin and sulfonamides.

  • NSAIDs

Non-steroidal anti-inflammatory drugs are used in treating fever and swelling. Some popular NSAIDs include naproxen and ibuprofen.

  • Seizure medications

These drugs are used in managing seizures and convulsions.

  • Diuretics

Diuretics are used in increasing the amount of urine excreted by the body.

Other factors can likewise cause SJS, and these are influenza, hepatitis, HIV, Diphtheria, Herpes and typhoid, to name a few.

Steven Johnson Syndrome symptoms

It is very important for parents to be vigilant of the manifestations of SJS in order to take the prompt actions to prevent the syndrome from getting worse. Among the symptoms to watch out for include:

  • Flu-like symptoms

Children with SJS feel that they have a flu, which is characterized by fever, chills, cough, sore throat and headache.

  • Skin rashes and lesions

These are manifested as purple or reddish rashes on the skin which quickly spread to the various body parts. Lesions are evident on the eyes, nose and mouth as the upper skin layer is slowly shed off.

  • Swelling

They may be evident on the face and tongue. This is extremely dangerous if it occurs in air passages like the bronchus which can suffocate the patient.

Symptoms of SJS are more pronounced when the syndrome has already advanced. During this time, the inflammation has spread and become visible on the skin. The syndrome may also give rise to disabling and life-threatening complications which include:

  • Vision problems
  • Secondary skin disease
  • Permanent skin problems
  • Sepsis
  • Internal organ damage

Steven Johnson Syndrome Diagnosis

Doctors diagnose SJS based on the medical history of the patient, taking into special consideration the medications he/she has been taking. Physical examination is also done to determine the severity of the symptoms. Skin biopsy, as well as other diagnostic tests like X-rays and blood tests, may also be performed.

Treatment for Steven Johnson Syndrome

The treatment aims to avoid the dreadful consequences of the disease by addressing the underlying cause and prevent it from aggravating. Aside from immediately eliminating the medication that caused the allergic reaction, meticulous skin care and fluid replacement may also be needed.

The chances of recuperation differ with every patient, depending on the severity of the condition. Those suffering from mild SJS will pull through within 1 to 2 months. However, the recovery period for those with severe SJS may take longer or not happen at all.

Steven Johnson Syndrome pictures


 

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