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Stevens-Johnson syndrome

Definition


Disease: Stevens-Johnson syndrome Stevens-Johnson syndrome
Category: Allergies
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Disease Definition:

When someone’s skin and mucous membranes react severely to a medication or infection, it means that they have a disease called Stevens-Johnson syndrome, which is a rare but serious disease. Initially, this syndrome produces flu-like symptoms, which are followed by blisters and a painful red or purplish rash that spreads, ultimately causing the top layer of the skin to die and shed.

Eliminating the underlying cause, controlling symptoms and minimizing complications is what the treatment for Stevens-Johnson syndrome will aim at. This syndrome, which usually requires hospitalization, is considered a medical emergency.

Based on the severity of the condition, recovery can take either weeks or months. Additionally, if a medication was the cause of Stevens-Johnson syndrome, the patient should permanently avoid that medication, as well as any others that are related to it.

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

Some of the signs and symptoms of Stevens-Johnson syndrome may be:

-    Skin pain
-    Facial swelling
-    Hives
-    Shedding or sloughing of the skin
-    Tongue swelling
-    Blisters on the skin and mucous membranes, particularly in the eyes, mouth and nose
-    A rash that is red or purple in color, and which spreads within hours or days

In case someone has Stevens-Johnson syndrome, then cough, fever, sore throat and burning eyes are some of the symptoms that they may experience a few days before the rash starts developing.


If someone experiences any of the symptoms mentioned below, they should seek emergency medical care:

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

Causes:

In most cases, an allergic reaction in response to infection, illness or medication is the cause of Stevens-Johnson syndrome. However, its exact cause can’t always be found.

MEDICATION CAUSES:
In most cases, medications are the cause of Stevens-Johnson syndrome, such as anticonvulsants, which are used to treat seizures; NSAIDs (nonsteroidal anti-inflammatory drugs); anti-gout medications, such as allopurinol; penicillins and sulfonamides, which are used in treating infections.

INFECTIOUS CAUSES:
Typhoid, HIV, hepatitis, influenza, diphtheria and herpes (both herpes simplex and herpes zoster) are some of the infections that may end up causing Stevens-Johnson syndrome.

OTHER CAUSES:
Physical stimuli, such as UV light or radiation therapy, may cause Stevens-Johnson syndrome in some cases.

There isn't a test that can help predict who is at greater risk of this disease, which makes Stevens-Johnson syndrome unpredictable. This disease is rare, however, there are some factors that may increase the risk of developing it, such as:

Existing medical conditions:
Someone’s risk of developing Stevens-Johnson syndrome may increase if he/she has:

-    Viral infections
-    Systemic lupus erythematosus, which is a chronic inflammatory disease
-    HIV (human immunodeficiency virus)
-    Other diseases that may decrease immunity

Genetics:
The risk of developing this syndrome may also increase in case someone carries a gene called HLA-B12.

Complications

Complications:

Some of the complications that Stevens-Johnson syndrome may cause include:

Secondary skin infection (cellulitis):
Sepsis and meningitis, which is the infection of the membrane and fluid that surrounds the brain and spinal cord, are some of the life-threatening complications that this acute infection of the skin may cause.

Sepsis:
Sepsis, which is a rapidly progressing and life-threatening condition, may lead to shock and organ failure. When bacteria from a massive infection enter the bloodstream and spread throughout the body, sepsis occurs.

Eye problems:
Inflammation in the eyes may be the result of the rash that this syndrome causes. Mild cases cause only irritation and dry eyes, while severe cases may lead to extensive tissue damage and scarring within the eyes, resulting in blindness.

Damage to internal organs:
A person may develop hepatitis, which is inflammation of the liver; myocarditis, inflammation of the heart; pneumonitis, of the lungs; and nephritis, of the kidney, due to the lesions of the internal organs caused by Stevens-Johnson syndrome.

Permanent skin damage:
The skin may have abnormal bumps and coloring (pigmentation) when it grows back following Stevens-Johnson syndrome. Scars may not go away, fingernails and toenails may not grow normally and the patient’s hair may fall out due to lasting skin problems.

Treatments:

In most cases, this syndrome requires hospitalization in a burn or intensive care unit.

HOW TO STOP MEDICATION CAUSES:
Discontinuing any of the medications that may be the cause of Stevens-Johnson syndrome is the initial and most important point. The patient may be recommended stopping all nonessential medications, because determining exactly which medication is causing this problem is quite difficult.

SUPPORTIVE CARE:
The supportive care that someone may receive while hospitalized for Stevens-Johnson syndrome may be:

Fluid replacement:
Replacing fluids is quite important because skin loss may end up causing significant loss of fluid from the body. Fluids and nutrients may be given through a nasogastric tube, which is a tube that is placed through the nose and into the stomach.

Skin nursing:
In order to soothe blisters while they heal, cool and wet compresses may help. A dressing with a topical anesthetic may be placed over the affected areas, preceded by the removal of any dead skin.


MEDICATIONS:
Pain medications to reduce discomfort, antibiotics to control infection when needed, antihistamines to relieve itching and topical steroids to reduce skin inflammation are some of the commonly used medications in the treatment of Stevens-Johnson syndrome. Some of the other medications that the patient may receive are:

Intravenous corticosteroids:
When started within a day or two of the appearance of symptoms, these medications may shorten the period of recovery; additionally, they may reduce the severity of symptoms.

IGIV (immunoglobulin intravenous):
There are antibodies in this medication that may help the immune system stop the process of Stevens-Johnson syndrome.

However, the two methods mentioned above are still under study for the treatment of this syndrome.


SKIN GRAFTING:
In this method, the patient’s skin is removed from one area of the body and attached to another. In some rare cases, this method may be necessary to help the patient heal in case a large area of the body is affected by this disease.

Skin may start growing again within several days in case the underlying cause of Stevens-Johnson syndrome is eliminated and the skin reaction is halted. However, full recovery may take several months if the condition is severe.

Prognosis:

Not available

Expert's opinion

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