Steven Johnson Syndrome

Steven Johnson Syndrome, or simply abbreviated as SJS, is an allergic reaction that is caused by the skin, genital areas, conjunctiva and mucous membranes. It mostly occurs when you are hypersensitive to an allergic reaction that you are unpredictable of, caused by a certain medicine you have consumed. The disease may also be caused by an infection. It is a rare disease but poses a serious risk on the outside as well as inside of your body. It makes eating, swallowing, and even releasing urine hard. It is a medical emergency that often shows symptoms like flu, followed by a purplish or red rash on the skin. The soft tissue layer of a mucous membrane positioned in the digestive system from mouth to anus, as well as reproductive organs and eyeballs, gets affected. It spreads and forms blisters, causing pain. The skin that is affected ultimately dies and peels off. Usually, this condition requires hospitalization of the affected person. The patient would often need emergency treatment to be provided in intensive care or burns unit. It takes weeks, or even months to recover, depending upon the severity of the condition. But you don’t need to panic as there is a special team of doctors and nurses available in the hospital who can give you the best treatment they know.

Symptoms

There are several symptoms of Steven Johnson Syndrome that you will observe before and after the disease occurs. Fever and flu-like feeling occur during the initial stages. There are a severe headache and pain in the joint. You get a cough and fatigue as a result of fever temperature of 100.4F or above. After a few days, a red or purplish rash appears which spreads in hours or days. It looks lighter around the outside and darker in the middle. Large blisters develop on the skin and mucous membranes of your eyes, nose, mouth and genital. You may get watery and burning eyes and the surface of your eyes may also get affected, causing vision problem and corneal ulcers. Swallowing becomes difficult due to blistered and ulcerated mucous membranes in your mouth. This leads to serious problems, like dehydration. The blisters then burst to leave painful sores. Your skin starts to shed within days after blisters form. One of the common symptoms of this condition is the swelling of lips and face, covered in crusty sores. Steven Johnson Syndrome is that serious a systemic disorder that it can potentially cause severe morbidity and even death. A person suffering from this condition needs immediate medical attention and should see a doctor as soon as possible.

Causes

Steven Johnson Syndrome is a disease that occurs rarely with an unpredictable reaction. Usually, it is triggered by an infection or a medication.

Infectious causes

A person, especially children, becomes more prone to Steven Johnson Syndrome when triggered by an infection. Viral infections, such as pneumonia and herpes, which causes cold sores, can result in SJS. This mostly happens with kids than adults. There are several risk factors involved in this disease, such as:

-  Viral infections like hepatitis A, pneumonia, or herpes can trigger the disease.

-  Glandular fever caused by Epstein-Barr virus.

-  Bornholm disease caused by Coxsackie virus.

-  Bacterial infections

-  Flu

-  Mumps

-  The family history of Steven Johnson Syndrome may increase the risk of getting the disease if any family member has had the syndrome.

-  The previous history of Steven Johnson Syndrome may put you at the risk of recurrence if the same medication is taken again.

-  Chemotherapy, HIV/AIDS, organ transplant, or autoimmune conditions can weaken the immune system.

Medication causes

When the reaction is caused by medication, the disease starts to occur while you are using it, or may take up to two months to occur. There are several drugs that can cause SJS. Some of the common medicines that can generate this syndrome are:

-  Allopurinol, like Zyloprim and Aloprim, is anti-gout medications that are used in painful arthritis.

-  Kids may get infected with this disease on the usage of drugs that help to treat seizures or mental illness, like Carbatrol and Tegretol.

-  Drugs, like sulfa antibiotics that include Septra and Bactrim, are used to fight infections, which can cause SJS.

-  Pain relievers, such as naproxen sodium (Aleve), acetaminophen (Tylenol) and ibuprofen (Motrin and Advil) can also cause the disease.

Ignoring the infection or continuing to take medications that might have caused the syndrome will only cause adverse effects. You must consult a specialist to get treatment as early as possible in order to help protect our skin and other organs from getting damaged that could last for a longer period of time.

How is it diagnosed?

Steven Johnson Syndrome is usually diagnosed after 1 to 3 weeks of the characteristic rash being exposed to a known stimulus. A dermatologist is a skin specialist who will help you to know the causes of Steven Johnson Syndrome and provide you with treatments to heal your disease. The syndrome is diagnosed using certain tests and procedures:

-  Symptoms that could relate to SJS.

-  Physical examination, in which doctors usually identify your condition based on your previous medical reports.

-  A sample of your skin is removed for skin biopsy (laboratory testing) in order to confirm the diagnosis and confirm other causes that could be possible.

-  To rule out infection, the dermatologist may take skin or oral culture, or culture from other areas.

-  You may have to undergo a chest X-ray, as referred by the doctor, to check for pneumonia.

-  Depending on your symptoms, you might be asked to get the required blood tests done so as to confirm infection or possibly other causes.

-  When 30 percent of the body is spread with rash or other symptoms of Steven Johnson Syndrome, the condition is called as TENS.

-  People diagnosed with TENS condition are at 20-30 percent higher risk of death as compared to the patients with SJS.

Prevention: What is the preventive measure for Steven Johnson Syndrome?

The answer is, there are several factors that need to be considered for preventing yourself completely from the disease.

-  Though carbamazepine, i.e. Carbatrol and Tegretol, is useful in treating bipolar disorder, epilepsy and other mental conditions, you must talk to your doctor before taking it. It is because, people with the HLA-B*1502 gene, who happens to be taking this drug, are at greater risk of causing Steven Johnson Syndrome.

-  In case of genetic susceptibility in your family, the members may also have to avoid using the medication. Drugs, such as allopurinol and carbamazepine, are associated to pose risk for Steven Johnson Syndrome. You will need to undergo genetic testing before taking these medications. This helps to determine whether you carry HLA-B*1502 gene.

-  As there is the chance of recurrence, the doctor might warn you to look out for the symptoms to prevent yourself from getting it again in future. You will need to avoid the medication and other therapy to prevent the chance of recurrence. It is because the severity of repeated occurrence of this disease could be more fatal than the previous episode.

Treatment:

In case you suspect to have signs of Steven Johnson Syndrome in you or your child, then you must seek emergency medical care. Avoiding the symptoms would possibly become severe and life-threatening. Such conditions are required to be treated in burn unit or intensive care unit. The doctor will recommend you to discontinue taking medications, possibly for the rest of your life, that are determined to cause Steven Johnson syndrome. Your doctor may advise you to stop taking all nonessential drugs as it is difficult to identify which drug has exactly caused the syndrome. Treatment will be started to provide relief from symptoms. These include several stages:

-  Strong painkillers will be given to ease the pain of skin areas that have been affected.

-  The dead skin is removed gently and cool, moist bandages are held over the affected area.

-  It is then applied with unscented, plain moisturizer on regular basis.

-  A nasogastric tube is inserted through your nose and into the stomach to let the nutrition and fluids pass through it.

-  An antiseptic or anaesthetic mouthwash helps to numb your mouth temporarily so that you can swallow easily.

-  Your specialist will advise you to take a short course of topical corticosteroids tablets in order to control the inflammation on the skin.

-  On suspecting poisoning in the blood (sepsis), antibiotics are given.

-  In case of eye-related symptoms, you will be given eye drops or ointment for eyes.

The time taken by the patient to recover from Steven Johnson Syndrome depends upon the severity of the condition. It might take weeks, or even months, to completely recover. The reactions on the skin will stop once the cause of this disease is treated successfully in the case of infection, or stopped in medication case. Within several days a new layer of skin may start to grow.

Complications:

A lot of complications occur if prevention is not taken on time. The disease includes a number of complications caused due to adverse effects on the skin and mucous membranes. These are:

-  Change in skin colour with scar appearing on it, as the skin grows back with uneven colour.

-  Cellulitis, a secondary skin infection, which further leads to serious issues like sepsis (blood poisoning).

-  Internal organs become inflamed. For instance, kidneys (nephritis), lungs (pneumonia), liver (hepatitis), or heart (myocarditis). A narrowed and scarred oesophagus (oesophageal stricture) may also occur.

-  In mild rash, your eyes may cause irritation and dryness. In the case of severe rash, your eyes may cause corneal ulceration, uveitis (inflammation of the middle layer of eyes), and even blindness.

-  Narrowing of the vagina due to scar tissue growing there and a scar on the penis are also possible due to Steven Johnson Syndrome.

Myths:

Myth #1: There are several misconceptions related to Steven Johnson Syndrome. If the treatment is not provided on time, the disease can be fatal and even cause death. You need to get rid of the common myths regarding this condition and know the actual facts.

Myth #2: There is a common misconception that Steven Johnson Syndrome is an allergic reaction, can be passed on to anyone. The fact is it cannot be passed even through direct contact. Many people these days are suffering from allergies caused by food and environment. Cold like symptoms develops from eating a certain food item, or while enjoying a warm spring day. These can last for hours, even days. Because of such environmental condition, the person with SJS is often admitted to ICU or burn units to limit his contact with bacteria, virus, or any other germs that could possibly trigger the disease, and not because there is a risk of passing the allergy or reaction.

Myth #3: Another myth which is doing rounds is that you can stop taking prescribed medicine when the allergic reactions have recovered. No, do not discontinue taking medicines prescribed by your doctor as and when you feel like. You just need to check with your doctor first. There are chances that the disease develops within the first two months of beginning a new medicine prescribed. If you have not yet suffered a reaction, it is not likely to appear later.

Myth #4: It is believed that the syndrome occurs only in adults and has no chances to affect children. The truth is it can pose harm to kids as young as 3 months old, or adults aged 50 years old.

Conclusion:

It can be concluded that Steven Johnson Syndrome is a rare medical condition that appears like severe burns and blisters caused by infection and toxic reaction to medications that includes painkillers and antibiotics. Avoiding the signs and symptoms could only worsen your condition, with the possibility of even death. It is important to talk to a skin specialist about the conditions that you have incurred and do as they assist you. Know about the causes of this syndrome and get treatment as early as possible. This would help you to recover sooner and prevent you from adverse situations. If you or someone close in your family has contracted this disease, then immediately consult a dermatologist near you to avail suggested treatments.

Last updated on : 14-09-2018

Disclaimer : Docprime doesn’t endorse or take any guarantee of the accuracy or completeness of information provided under this article and these are the views strictly of the writer. Docprime shall not be held responsible for any aspect of healthcare services administered with the information provided on this article.

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