Does your skin suddenly turn red, blister, and peel? Let's learn about this dangerous Stevens-Johnson Syndrome (SJS)?

Have you ever seen, or has it happened to you, a sudden rash on your body that turns red, looks like a rash, then blisters appear and the skin peels off? These lesions can also appear inside the mouth, in the eyes, and in the genital area. This is not a simple thing, and can be a serious condition that requires immediate hospitalization . Today, we will talk about this condition called Stevens-Johnson Syndrome (SJS).

What is Stevens-Johnson Syndrome (SJS)?

Simply put, Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are two very serious skin conditions. What happens is that your skin suddenly starts to develop red, itchy bumps, which then blister and peel off. It can also affect your eyes, genitals, and the mucous membranes inside your mouth. If you develop these conditions, you will definitely need to be hospitalized for treatment.

Some doctors consider SJS and TEN to be two separate diseases, while others say they are different stages of the same disease. SJS is a slightly less severe form of the disease than TEN. For example, in SJS, less than 10% of the skin on the entire body may be lost. In TEN, more than 30% of the skin may be lost. However, it is important to remember that both conditions can be life-threatening .

What other names does SJS have?

Yes, this is called by several other names. For example:

• Lyell's Syndrome
• Stevens-Johnson syndrome/toxic epidermal necrolysis
• Stevens-Johnson syndrome toxic epidermal necrolysis spectrum

Sometimes, the name changes depending on the cause. If it is caused by a certain drug, it can be called ``drug-induced Stevens-Johnson syndrome'', and if it is caused by an infection called ``Mycoplasma'', it can be called ``mycoplasma-induced Stevens-Johnson syndrome''.

Who is more likely to develop SJS?

SJS is most often seen in young people under the age of 30 and in young children. However, it can occur in people of any age, especially the elderly. Women are more likely to develop SJS than men.

Imagine, if a young child gets an infection like pneumonia and then develops a skin problem like this... it could be SJS. The main cause of SJS in children is infections like pneumonia. But in adults, SJS/TEN is mostly caused by the medications they take.

What are the risk factors for developing SJS?

There is not a single cause for this disease, but a combination of factors. The most important of these is genetic predisposition. That is, your genes may have some influence. Certain environmental factors can also activate those genes. One of these genetic factors is the special parts of our body called `(Human Leukocyte Antigens - HLAs). People with certain variants of these `(HLAs)` are at a higher risk of developing SJS or TEN than others.

What are the symptoms of SJS?

There are several symptoms that can occur in SJS. It is important to be aware of these:

• Severe pain in the skin: First, the skin starts to hurt.
• Fever: You have a fever.
• Body pain: Feels like your whole body hurts.
• Red spots or patches on the skin: Red spots or patches appear on the skin.
• Cough.
• Blisters and sores: Blisters and sores can occur not only on the skin, but also on the mucous membranes in places like the mouth, throat, eyes, genitals, and rectum. Imagine, sores inside the mouth make it difficult not only to eat but also to talk.
• Peeling of the skin: The skin peels off from the blisters.
• Drooling: Due to mouth sores, saliva comes out when you can't keep your mouth shut.
• Eyelid swelling: Blisters and swelling in the eyes cause the eyes to become so swollen that they cannot be opened.
• Pain during urination: Blisters in the mucous membranes cause severe pain during urination.

Imagine you started a new medication, and within a few days you feel unwell, have a fever, and aches, and then suddenly red spots appear on your skin and blisters appear... That's when you should start to suspect that this is SJS.

What causes SJS?

There are several main causes of SJS:

• Allergic reaction to a drug: This is the main cause of most SJS and all TEN cases. This can happen if you miss a dose of a medication you are taking.
• Infections: For example, infections such as Mycoplasma pneumonia, Herpes virus, and Hepatitis A.
• Graft-versus-host disease: This is a condition that can occur, especially after a bone marrow transplant.
• No cause found: Sometimes SJS can develop without any obvious cause.

If you have SJS because of a drug, symptoms usually start to appear within one, two, or three weeks of starting the drug. You may start with a fever, cough, and headache, followed by skin rashes and peeling. In some people with TEN, hair and nails may even fall out. Very rarely, SJS has been reported to be caused by a recent vaccination.

What medications are most likely to cause SJS?

There are certain medications that have a higher risk of causing SJS. These include:

• Antibacterial sulfa drugs.
• Anti-epileptic drugs: Examples include Phenytoin (Dilantin®), Carbamazepine (Tegretol®), Lamotrigine (Lamictal®), and Phenobarbital (Luminal®).
• `(Allopurinol (Aloprim®, Zyloprim®))`: This is a medicine used for gout and kidney stones.
• Non-steroidal anti-inflammatory drugs (NSAIDs): Examples include Piroxicam (Feldene®), Nevirapine (Viramune®), and Diclofenac (Cambia®, Flector®).
• Other antibiotics.

Important: This does not mean that everyone who takes these medications will develop SJS. However, these medications have been found to be associated with an increased risk of developing SJS.

Are there other factors that increase the risk of developing SJS?

Yes, there are some other conditions that increase the risk of developing SJS. They are:

• Having had a bone marrow transplant.
• Having autoimmune diseases such as `(Systemic lupus erythematosus - SLE)`.
• Human immunodeficiency virus (HIV) infection.
• Other chronic diseases of joints and connective tissue.
• Cancer.
• Having a weakened immune system.
• If someone in your family has previously developed SJS (family history).
• Having a variant of a specific gene called ``Human Leukocyte Antigen-B - HLA-B''.

How do doctors diagnose this SJS condition?

Doctors diagnose SJS and TEN by considering these factors:

• Examination of the affected skin and mucous membranes: Usually at least two mucous membranes are affected.
• Depending on the level of pain you feel.
• Depending on how quickly your skin was affected.
• Depending on how much your skin is affected.
• Skin biopsy: Sometimes, a small sample of skin is taken and examined under a microscope to confirm the diagnosis.

How is SJS treated?

SJS is a condition that requires immediate hospitalization and treatment. Trying to treat it at home can make the condition worse. The following are some of the treatments:

• Immediately stop the medication that is thought to be causing the problem: This is the first thing to do.
• Intravenous (IV) fluids to restore electrolytes: This is done to control the loss of fluid from the body as the skin peels off.
• Applying non-adhesive dressings to the affected skin.
• Giving high-calorie foods to speed up recovery: Sometimes, tube-feeding may be necessary.
• Administering antibiotics if necessary to prevent infection.
• Giving painkillers.
• Treatment in a hospital, possibly in an Intensive Care Unit (ICU) or Burn Unit.
• Seeking help from specialist teams such as Dermatology and Ophthalmology (if the eyes are affected).
• In some cases, specific treatments such as IV immunoglobulin, cyclosporine, IV steroids, or amniotic membrane grafts (for the eyes) may be given.

What are the possible complications of SJS?

Because SJS and TEN are serious conditions, complications can be severe.

• Death: This is the most serious complication. About 10% of patients with SJS and about 50% of patients with TEN can die.
• Pneumonia.
• Sepsis: This is a severe bacterial infection, similar to blood poisoning.
• Shock state `(Shock)`.
• Multiple organ failure.

It is because of these severe complications that SJS/TEN conditions require immediate hospitalization.

Can SJS be prevented?

In fact, in most cases, SJS cannot be prevented. Because it is often caused by an unexpected reaction to a medication, there is no way to know if this will happen before taking a medication.

However, if you are diagnosed with a drug-induced condition, the most important thing is to never use that drug or a similar class of drugs again. It is very important to inform your doctor about this and to record it in your medical records.

What is the outlook for someone with SJS? (Outlook)

Not everyone with SJS experiences the same symptoms. Some people recover within a few weeks. However, if symptoms are severe, it can take months to fully recover. Some people may experience long-term effects. For example:

• Skin: Dry skin, itching, skin discoloration.
• Eyes: Frequent swelling and/or dryness, frequent eye inflammation, blurred vision, sensitivity to light (photophobia).
• Excessive sweating.
• Lung damage: Conditions such as chronic obstructive pulmonary disease (COPD), asthma.
• Nails falling off or becoming deformed.
• Hair loss (Alopecia).
• Dryness of the mucous membranes: This can cause things like difficulty urinating.
• Chronic fatigue syndrome.
• Changes in taste.

Importantly, the disease can recur if the person is re-exposed to the drug that caused the SJS in the first place. If that happens, the symptoms are usually more severe the second time around.

About 10% of SJS patients and 30% of TEN patients die, mainly due to sepsis, acute respiratory distress syndrome (ARDS), and multiple organ failure.

Here are some things you should remember from what we've talked about (Take-Home Message):

Okay, so we've talked a lot about this dangerous condition called SJS. Here are some things you need to remember:

• Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are very serious, even life-threatening skin conditions.
• If you develop symptoms such as skin pain, red spots, blisters, peeling skin, or mouth or eye sores after starting a new medication, especially within a week or two, seek medical advice immediately. It could be SJS.
• Always tell your doctor if you have had an allergy or severe adverse reaction to a medication before.
• Never use a drug or similar drugs that have been identified as causing SJS again.**
• If you suspect this condition, don't stay home, go to a hospital immediately. Quick treatment can save your life.

I hope this information is useful to you. Stay healthy!

` Stevens-Johnson Syndrome, SJS, Toxic Epidermal Necrolysis, TEN, Skin diseases, Allergies, Drug side effects, Blisters, Skin rashes, Dermatological diseases