What Is Stevens Johnson Syndrome?

When faced with Stevens-Johnson Syndrome, which has the potential to be a very distressing and life-altering condition, it's completely understandable to feel scared and overwhelmed.

A rare but serious disorder of the skin and mucous membranes,Stevens-Johnson syndrome (SJS) frequently results from an allergic reaction to drugs or infections. It can begin with symptoms resembling the flu, and develop into a painful rash that spreads and blisters, causing the skin's top layer to peel off.¹

Continue reading to discover more about SJS's risk factors, symptoms, and prevention, as well as its most recent studies and available treatments.

Overview

Stevens-Johnson syndrome (SJS) is a rare and serious condition of the skin and mucous membranes. It typically manifests as a bad reaction to certain medications with flu-like symptoms at first, followed by a painful rash that spreads and blisters. After a few days, the top layer of the affected skin dies, sheds, and starts to heal.

Stevens-Johnson syndrome is a serious medical condition that usually requires hospitalisation. Eliminating the cause, treating wounds, managing pain, and preventing complications as new skin grows are the main goals of treatment. The recovery process can take weeks or months.

The condition may also be classified as toxic epidermal necrolysis (TEN), which is a more severe variation of SJS. TEN causes significant damage to the mucous membranes and involves more than 30% of the skin's surface.

Causes of stevens johnson syndrome

There are many potential causes of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), although infections and allergic reactions to specific medicines are the most frequent. Medications that may induce SJS/TEN include:

• Antibiotics (such as sulfa drugs)
• Non-steroidal anti-inflammatory drugs (NSAIDs)
• Anticonvulsants
• Some chemotherapy drugs 

Apart from reactions to medications, there are some illnesses and bacterial infections that can cause this disease, including, but not limited to:

•  Herpes simplex virus
•  Mycoplasma pneumonia

Certain genetic markers have been identified as potential risk factors, and in some circumstances, there may be a genetic predisposition to developing SJS/TEN. A history of autoimmune diseases, being elderly, and having a weakened immune system are additional risk factors for developing SJS/TEN.¹

Signs and symptoms of stevens johnson syndrome

Similar symptoms are present in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), but the severity of skin detachment varies.

Flu-like symptoms such as fever, sore throat, cough, and body aches are among the first signs of SJS. These symptoms are usually accompanied by a painful rash on the face, trunk, and extremities, which appears within a few days. The rash may begin as small red or purple spots that spread and merge to form large, flat, blistery areas. Blisters can be fluid-filled and easily rupture, leaving painful, raw, and exposed skin behind.

As SJS progresses, the affected skin and mucous membranes can become increasingly painful and inflamed. Lips, mouth, and throat can develop painful sores and ulcers, making it difficult to eat, drink, or talk. The eyes can also be affected, resulting in redness, pain, and discharge. In severe cases of TEN, more than 30% of the skin can be affected, resulting in significant fluid loss, electrolyte imbalances, and even sepsis, a potentially fatal condition that occurs when the body's response to infection damages its tissues and organs.²

If you suspect that you or a loved one has SJS or TEN, seek medical attention immediately. A dermatologist or an emergency room doctor can diagnose the condition based on a physical examination and a review of your medical history, medications, and recent illnesses. Prompt and aggressive treatment is required to avoid complications and improve outcomes. 

Management and treatment for stevens johnson syndrome

SJS/TEN management and treatment can be a complicated process that often necessitates hospitalisation. Once the offending medication or cause has been identified, it is critical to discontinue its use immediately. Patients may also require supportive care, such as intravenous (IV) fluids and electrolyte replacement because the condition can cause dehydration and electrolyte imbalances. Pain relief is also required because the condition can be excruciatingly painful.

Patients will need to have their skin treated to prevent further damage and infection. This is usually accomplished by applying an antibiotic ointment to the affected areas of skin and dressing them with non-adhesive dressings. More severe cases may necessitate skin grafts or surgery to remove damaged skin. Ophthalmologic (eye) care is also required in SJS/TEN patients because it can cause severe eye complications that can result in permanent vision loss.

SJS/TEN prevention can be difficult because the exact cause is not always known. However, potential risk factors such as specific medications, viral infections, and autoimmune diseases must be considered. If you are starting a new medication, it is critical to be on the lookout for SJS/TEN symptoms such as rash, fever, and blistering. Seek medical attention right away if you suspect you have SJS/TEN.³

Regarding prevalence, SJS/TEN is considered a rare condition, affecting about 1-2 people per million each year. However, the prevalence may be higher in certain populations, such as those suffering from autoimmune diseases or taking certain medications.⁴

Diagnosis of stevens johnson syndrome

Stevens-Johnson Syndrome can be difficult to diagnose because its early symptoms are non-specific and mimic those of other common infections. However, early detection is essential for preventing the condition from worsening and causing serious complications. Doctors use the following techniques to diagnose SJS:

• Medical History and Physical Exam. A thorough medical history and physical exam are the first steps in diagnosing SJS. The doctor will inquire about the patient's medications, recent illnesses or infections, and examine the skin and mucous membranes for rashes, blisters, or lesions
• A skin biopsy is the removal of a small sample of the affected skin for microscopic examination. This aids in the confirmation of the diagnosis and the exclusion of other skin disorders
• Blood tests can help determine the extent of SJS damage and detect any underlying infections or inflammation
• An eye examination is required, if the eyes are affected, to check for corneal ulcers, inflammation, and scarring
• Allergy testing may be performed to determine the cause of the reaction that caused SJS
• Testing for an infection. To rule out an infection, your doctor will take a skin, tissue or fluid sample and test it for the presence of bacteria or viruses
• Imaging. Depending on your symptoms, your doctor may order imaging tests, such as a chest X-ray, to rule out pneumonia⁵

Risk factors of stevens johnson syndrome

Although the precise cause of SJS/TEN is unknown, some known risk factors can increase the likelihood of developing the condition:

• Medications: Antibiotics, anti-inflammatory drugs, anticonvulsants, and antiretroviral drugs have all been linked to SJS/TEN
• Infections: Viral and bacterial infections, such as herpes, influenza, and pneumonia, can activate an immune response that results in SJS/TEN
• Genetic predisposition: Some people may be predisposed genetically to developing SJS/TEN, though more research is needed to fully understand this relationship
• Age and gender: SJS/TEN can affect people of any age or gender, but some research suggests that older people and women are at a slightly higher risk
• Previous SJS/TEN diagnoses: Individuals who have previously been diagnosed with SJS/TEN are at a higher risk of developing the condition again

It is important to note that, while these risk factors can increase the likelihood of developing SJS/TEN, not everyone who meets these criteria will develop the condition. If you are taking medication or have a known risk factor for SJS/TEN, it is important to be aware of the condition's signs and symptoms and to seek medical attention immediately if you develop any of these symptoms.⁶

Complications of stevens johnson syndrome

SJS and TEN can lead to serious complications, some of which are fatal. SJS/TEN complications include the following:⁷

• Sepsis: sepsis is a potentially fatal complication that occurs when the body's immune system overreacts to an infection, resulting in widespread inflammation and organ damage
• Dehydration: dehydration can occur as a result of fluid loss from skin peeling and blistering
• Vision loss: eye damage can cause vision problems or even blindness
• Scarring: scarring can result from skin damage, particularly if the affected person scratches or picks at the affected areas
• Infection: open sores can become infected, leading to additional complications
• Breathing difficulties: SJS/TEN can affect the respiratory system in rare cases, causing breathing difficulties
• Arthritis:  SJS/TEN can cause joint pain and stiffness, which can lead to long-term joint problems

Summary

Stevens-Johnson Syndrome (SJS) is a rare and frightening skin condition that can affect anyone of any age or gender. It can be caused by a variety of factors, such as infections, medications, and autoimmune diseases. SJS is a serious condition that requires immediate medical attention, but it is manageable with proper care. It is critical to be aware of any medications or substances that may cause SJS so that you can take the necessary precautions to avoid them. If you have any of the symptoms listed above, you should immediately seek medical attention. Remember that early intervention can make a big difference in how this condition turns out.