Steven's syndrome

Steven-Johnson Syndrome (SJS) is a disease that is quite rare in Indonesia, but is a serious condition. This disease causes the sufferer's skin to itch, blister, and even peel as a result of an overreaction to certain drugs and infections.

People with Stevens-Johnson Syndrome must be rushed to the hospital for treatment, while the recovery period can take weeks. Even if the symptoms are very severe, this disease can lead to death. Read on to learn more about Steven-Johnson syndrome.

What is Steven-Johnson syndrome?

Stevens-Johnson syndrome is a rare syndrome (symptom collection) that occurs when the skin and mucous membranes overreact to a drug or infection. The mucous membrane is the inner skin layer that lines the various body cavities that have contact with the external environment and internal organs of the body. In some parts of the body, mucous membranes are attached to the skin, for example the nostrils, lips, inner cheeks, ears, pubic area, and anus.

What are the signs and symptoms of Steven-Johnson syndrome?

This syndrome begins with flu-like symptoms such as fever, cough, hot eyes, and sore throat. But after a few days there will be a red or purplish rash on the skin that feels sore and spreads or even blisters, joint pain, to swelling of the face and tongue. In many cases, the cells in the outermost layer of skin die and the skin begins to peel off.

What are the causes of Steven-Johnson syndrome?

This rare syndrome is generally triggered by drug use. There are several types of drugs that most often trigger Steven-Johnson syndrome, including the following:

• Anti-gout medications, eg allopurinol
• Non-Steroid Anti-Inflammatory Drugs (NSAIDs) which are widely used to relieve pain, for example mefenamic acid, ibuprofen, salicylic acid, piroxicam
• Antibiotic drugs, especially Penicillin
• Seizure medication, usually used by people with epilepsy.

However, the symptoms of Steven-Johnson in some people can also be triggered by infection with certain viruses or germs, including the following.

• Herpes (herpes simplex or herpes zoster)
• Influenza
• HIV
• Diphtheria
• Typhoid
• Hepatitis A
• Pneumonia

In certain cases, Stevens-Johnson Syndrome can also be triggered by physical stimuli such as radiotherapy and ultraviolet light. But sometimes, the exact cause is not always certain, making it difficult to prevent.

What are the possible complications of Steven-Johnson syndrome?

Some of the complications that will arise due to Steven-Johnson syndrome include:

• Secondary skin infection (cellulitis). Cellulitis can cause life-threatening complications, including sepsis.
• Blood infection (sepsis). Sepsis occurs when bacteria from an infection enter your bloodstream and spread throughout your body. Sepsis is a fast-growing and life-threatening condition that can lead to perfusion and organ failure.
• Eye problems. The rash caused by Stevens-Johnson syndrome can also cause inflammation in your eye. In mild cases, this syndrome can cause irritation and dry eyes. In severe cases, it can cause extensive tissue damage and scar tissue that causes visual disturbances and even blindness.
• Lung involvement. This condition can cause acute respiratory failure.
• Permanent skin damage. When your skin grows back, there may be skin you won't be able to 100 percent of like all. Generally there are lumps, discoloration, and very possibly will cause scars. Apart from skin problems, this syndrome will also cause your hair to fall out, and your fingernails and toenails may not grow normally.

How is Stevens-Johnson syndrome treated?

The first aid for treating drug allergy in Stevens-Johnson syndrome is to stop taking the drug that triggers the allergy. Furthermore, people with Steve Johnson syndrome must be taken to the hospital for intensive care.

Some of the drugs that doctors usually give to treat Steven-Johnson syndrome are giving allergy drugs (antihistamines) to relieve symptoms, or corticosteroids to control inflammation that occurs if the symptoms are severe enough.

In addition, supportive therapy given in the hospital includes rehydration or replacement of lost body fluids using intravenous drips. If a wound occurs, the dead skin should be cleaned and then the wound covered with a bandage to prevent infection.

How can you prevent Steven-Johnson syndrome?

There are several steps you can take to prevent this rare syndrome, namely:

• Generally for Asian people, it is recommended to carry out genetic testing before taking certain drugs such as carbamzepine.
• Consult a doctor if you do have a history of this disease.
• Avoid taking drugs that can trigger a relapse if you have previously had Steven-Johnson syndrome.