Stevens-Johnson Syndrome: Causes, Symptoms, Risk Factors, Diagnosis, Treatment and Complications

Stevens-Johnson syndrome, or SJS, is a rare but fatal disease that affects the skin and mucous membranes. It often starts out with flu-like symptoms before developing into a painful rash that spreads and blisters as a medication reaction. The top layer of the affected skin eventually dies, sheds, and then begins to heal after a few days.

Hospitalization is usually required in cases of medical emergencies like Stevens-Johnson syndrome. The aim of treatment is to get rid of the cause, heal wounds, control pain, and lessen complications as new skin develops.

Cause of Stevens-Johnson Syndrome

Stevens-Johnson syndrome is an uncommon and unpredictable disease. Your doctor may not always be able to pinpoint the exact cause of the illness, although it is often caused by a medication, an infection, or both. Some people may experience reactions while taking drugs or for up to two weeks after discontinuing them.

The following are medicines that can cause Stevens-Johnson syndrome:

• Anti-gout medication allopurinol is used.
• Medicines used to treat seizures and mental illness include anticonvulsants and antipsychotics.
• Antimicrobial sulfonamides, such as sulfasalazine
• Viramune (also known as Viramune XR) nevirapine
• Naproxen sodium (Aleve), ibuprofen (Advil, Motrin IB, etc.), and acetaminophen (Tylenol, etc.) are examples of pain relievers.
• Stevens-Johnson syndrome can develop from HIV and pneumonia, among other conditions.

Symptoms of Stevens-Johnson syndrome

One to three days before to the appearance of a rash, you may present with early Stevens-Johnson syndrome symptoms, such as:

• Throat and mouth soreness
• Fever
• Tired
• Burning eyes

The following are other signs and symptoms of the illness:

a rash that is red or purple and creates blisters on the skin, mucous membranes around the mouth, nose, eyes, and genitalia, as well as severe, unexplained cutaneous agony.

Several days after blisters form, the skin starts to slough.

Risk Factors Of Stevens-Johnson Syndrome

Some of the risk factors for stevens johnson syndrome are mentioned below:

• Infection with HIV. Stevens-Johnson syndrome is more common among HIV-positive individuals than the general population by a factor of roughly 100.
• A compromised immune system. HIV/AIDS, autoimmune illnesses, and organ transplants can all have an impact on the immune system.
• Cancer. Stevens-Johnson syndrome is more likely to affect those who have cancer, particularly blood malignancy.
• The condition of Stevens-Johnson's past. You run the danger of a recurrence if you use that medication again if you've experienced a medication-related variant of this ailment.
• Stevens-Johnson syndrome runs in the family. You may have a higher risk of developing Stevens-Johnson syndrome if a close blood relative has it.
• Genetic factors. In particular, if you also take medication for gout, seizures, or mental illness, having specific genetic variants increases your risk of developing Stevens-Johnson syndrome.

Diagnosis of Stevens-Johnson syndrome 

Stevens-Johnson syndrome is diagnosed using a variety of tests and methods, such as:

• A physical examination and a review of your medical background. A physical examination, a review of your recent and current medications, and your medical history can often help a doctor diagnose Stevens-Johnson syndrome.
• A skin biopsy. Your healthcare provider takes a sample of skin for laboratory analysis (biopsy) to confirm the diagnosis and rule out alternative explanations.
• Culture. Your doctor collects a sample of skin, tissue, or fluid for culture testing in the lab to rule out an infection.
• Imaging. Your healthcare practitioner may order imaging, such as a chest X-ray, to check for pneumonia based on your symptoms.
• Blood test. These are utilised to verify infection or any other cause which is possible

Treatment Of Stevens-Johnson Syndrome

Stevens-Johnson syndrome requires hospitalization for treatment that also possibly in an intensive care unit as it is a critical condition to treat.

Stopping any drugs that might be contributing to Stevens-Johnson syndrome is the first and most crucial step in the healing process.It could be difficult to pinpoint the problem drug if you take many drugs. Therefore, your doctor might urge you to stop taking any medications that aren't necessary.

While inpatient, you could get the following supportive care types:

• Food and fluid replacement. Since skin loss can cause your body to lose a significant quantity of fluid, fluid replacement is an essential part of treatment.
• You may be given fluids and nutrition via a tube (nasogastric tube), which is inserted into the nose and passed to the stomach.
• Wound treatment. Utilizing cool, wet compresses could lessen blister discomfort while they heal. Before applying Vaseline or another sort of therapeutic therapy to the problematic areas, your medical experts could carefully remove any dead skin.
• Therapy for the eyes. Ophthalmologists are doctors who specialise in treating diseases of the eyes.

Medications

The following drugs are used to treat Stevens-Johnson syndrome:

• Painkillers to lessen suffering.
• Medicine (topical steroids) for the management of inflammation of the eyes and mucous membranes.
• Medications to control infection as required.
• Other medicines that are taken orally or given systemically, like corticosteroids and intravenous immune globulin.
• Studies have shown that the medications cyclosporine (Neoral, Sandimmune) and etanercept (Enbrel) are successful in treating this illness.
• If the skin reaction is stopped and the Stevens-Johnson syndrome's underlying cause is treated, new skin may begin to develop within a few days. In extreme circumstances, full recovery could take months.

Complications of Stevens-Johnson syndrome 

Stevens-Johnson syndrome complications consist of:

• Dehydration. Fluid loss occurs at skin-shedding areas. Additionally, mouth ulcers and throat sores can make it difficult to take water, which can cause dehydration.
• Sepsis, also known as a blood infection. Sepsis is when bacteria from an illness spread throughout the body through the bloodstream. Sepsis is a rapidly progressing, deadly illness that may cause shock and organ failure.
• Issues with the eyes Stevens-Johnson syndrome's rash has the potential to irritate the eyes, dry them out, and make them sensitive to light.. Extreme situations could result in visual loss and, in rare cases, blindness.
• Lungs are involved. The disease may lead to acute respiratory failure, which happens when the lungs are unable to supply enough oxygen to the blood.
• Permanent skin injury. Your skin may regenerate after Stevens-Johnson syndrome with lumps and a darkened look (depigmentation). Perhaps you even have scars. Your fingernails and toenails might not grow as quickly as they once did, and your hair might start to fall out as a result of ongoing skin problems.

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