Imagine that you have a fever, body aches, and you start taking a medicine prescribed by a doctor. What if, within a few days, maybe a week or two, you suddenly develop red spots on your body, sores inside your mouth, in your eyes, and in your genitals, and your skin starts to blister and peel off like it was burned? This is truly a scary, very dangerous, and rare condition. Today we are going to talk about this condition called Stevens-Johnson Syndrome (SJS). It is very important for everyone to be aware of this.
What are SJS and TEN? What is the difference between the two?
Stevens-Johnson Syndrome (SJS) is a severe hypersensitivity reaction, usually to a drug. It involves damage to our skin and mucous membranes, causing the cells to die and the skin to peel off. Mucous membranes are the delicate, moist membranes that line our mouth, nose, eyes, genitals, and anus.
There is another severe form of this condition, which we call Toxic Epidermal Necrolysis (TEN) . Simply put, SJS and TEN are like two sides of the same disease.
- SJS (Stevens-Johnson Syndrome): In this case , less than 10% of the body's skin surface is sloughed off.
- TEN (Toxic Epidermal Necrolysis): This is a very serious and potentially life-threatening condition in which more than 30% of the body's skin is lost.
Both of these conditions are very serious, so the patient must be hospitalized , sometimes in an intensive care unit (ICU) or burn unit.
Who is most likely to get this disease?
Although SJS can occur at any age, it is most common in children and young adults under the age of 30. It can also occur in older people. SJS is slightly more common in women than in men.
There is a slight difference when looking at the reason:
- The main cause of SJS in children is infectious conditions such as pneumonia .
- The main cause of SJS/TEN in adults is an allergic reaction to a medication .
What exactly are the symptoms of SJS?
It is very important to be aware of these symptoms, because the sooner you recognize them, the sooner you can seek treatment. Symptoms usually appear within a week or three of starting a medication.
The first symptoms are common cold symptoms like fever, body aches, headache, and cough. This makes it difficult to diagnose SJS in the early days. Only then do severe skin symptoms appear.
Below are the main symptoms of SJS.
| Symptom | Simply explained |
|---|---|
| Severe skin pain | Feeling a burning pain so intense that you can't touch your skin. |
| Fever and body aches | Symptoms include high fever, body aches, and headaches. |
| Red spots and patches | Red spots or lesions that start on the face and chest and spread throughout the body. |
| Blisters and wounds | Blisters and painful sores on the skin, inside the mouth, throat, eyes, genitals, and rectum. |
| Skin peeling | The blisters burst and the top layer of skin peels off in large flakes. |
| Spitting out saliva from the mouth | Drooling due to mouth ulcers, inability to keep mouth closed. |
| Eye contact | Blisters and swelling in the eyes, making it difficult to open the eyes. |
| Pain during urination | Severe pain during urination due to blistering of the mucous membranes. |
Main causes and types of drugs that can cause SJS
The main cause of SJS is an allergic reaction to a certain drug. However, this does not happen with all drugs. There are several types of drugs that are more likely to cause SJS.
| Reason | Description |
|---|---|
| Allergies to medications | This is the cause of the majority of SJS and TEN cases. |
| Infections | Especially in children, it can be caused by infections such as Mycoplasma pneumonia, Herpes, and Hepatitis A. |
| Other reasons | Graft-versus-host disease, a complication that occurs after bone marrow transplants, vaccines (very rarely), and sometimes no cause can be found. |
Drugs with a higher risk of causing SJS
- Antibacterial sulfa drugs: Some types of antibiotics.
- Anti-epileptic drugs: For example, drugs such as `Phenytoin (Dilantin®)`, `Carbamazepine (Tegretol®)`, `Lamotrigine (Lamictal®)`, `Phenobarbital (Luminal®)`.
- Allopurinol: A drug used to treat gout and some types of kidney stones (`Aloprim®`, `Zyloprim®`).
- Painkillers (NSAIDs): Non-steroidal painkillers such as `Piroxicam (Feldene®)`, `Diclofenac (Cambia®, Flector®)`. Other medications such as `Nevirapine (Viramune®)`.
- Other Antibiotics: Some other types of antibiotics can also cause this.
Important: Not everyone will develop SJS from the medications listed here. Millions of people use them. However, these medications have been identified as having a higher risk of developing SJS. Never stop taking a medication prescribed by a doctor based on this article.
Diagnosis and treatment of the disease
If you or someone you know experiences the above symptoms, especially if you have just started a new medication, it is essential to immediately go to the Emergency Department (ETU) of the nearest hospital .
Doctors diagnose this disease by:
- By examining your skin and mucous membranes (such as mouth, eyes).
- By asking about the level of pain you are feeling.
- By looking at the rate at which skin symptoms spread and how much they have spread.
- If necessary, a small piece of skin can be taken and examined (skin biopsy).
Treatment for SJS
The main goals of treatment for SJS are to stop the drug that caused the disease, control symptoms, prevent complications, and help the skin recover. Hospitalization is mandatory for this.
Some treatment methods are listed below:
| Treatment method | Description |
|---|---|
| Discontinuation of the causative drug | This is the first and most important step. The drug suspected of causing SJS is immediately stopped. |
| Providing fluids and salts to the body | The skin peeling causes a large amount of fluid to be lost from the body, so IV fluids are used to provide the necessary fluids and electrolytes. |
| Skin treatment | The injured skin is covered with special non-adhesive dressings, just like treating a burn. |
| Providing nutrition | It is difficult to eat because of the mouth sores. Since it takes a lot of energy to heal, high-calorie food is given through a tube passed through the nose into the stomach (tube-feeding). |
| Pain control | This is a very painful condition, so strong painkillers are given. |
| Specialist medical service | Seek the help of a dermatologist and, if the eyes are affected, an ophthalmologist. |
Complications of SJS and effects after recovery
SJS is a very serious condition that can even lead to death. About 10% of patients with SJS and up to 50% of patients with the more severe form, TEN, die. The main causes of death are severe complications such as sepsis, pneumonia, and multiple organ failure.
Even if the disease is cured, some people may experience long-term effects.
- Skin changes: Dry skin, itching, skin discoloration.
- Eye problems: dry eyes, frequent eye discomfort, blurred vision, difficulty looking at light (photophobia).
- Other: Excessive sweating, lung damage, nail loss or deformity, hair loss, taste disturbances.
Very important: If you are re-exposed to the drug that caused SJS, the second time the condition can be much more severe than the first. Therefore, it is essential to remember the name of the drug that caused SJS and similar drugs for the rest of your life and to inform every doctor you see.
Take-Home Message
- Stevens-Johnson Syndrome (SJS) is a severe, life-threatening skin reaction, usually caused by a drug.
- Initial symptoms include fever and body aches, but later progress to painful red patches on the skin, blisters, and peeling skin.
- If you experience these symptoms after starting a new medication, go to a hospital's Emergency Department (ETU) immediately without delay.
- The main treatment is to identify and stop the drug that caused the disease, and provide supportive care in the hospital.
- Remember the name of the drug that caused your SJS for the rest of your life and inform your doctor about it every time you go for treatment. This could save your life.
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