A rare and serious condition known as Stevens-Johnson syndrome (SJS) affects the skin and mucous membranes, which line the mouth, nose, eyes, genitals, and anus. The disease can cause blisters, skin peeling, and tissue damage that can be life-threatening. It can be caused by medication, infection, or unknown factors.
The exact cause of SJS is not always known, but it is usually associated with an adverse reaction to a medication or an infection. Some of the medications that can cause SJS include:
Anti-gout drugs, such as allopurinol.
Anti-seizure and anti-psychotic drugs, such as carbamazepine and lamotrigine.
Antibiotics, especially sulfonamides, such as sulfamethoxazole and trimethoprim.
Pain relievers, such as acetaminophen, ibuprofen, and naproxen.
Anti-HIV drugs, such as nevirapine and abacavir.
Some of the infections that can cause SJS include:
Pneumonia, caused by bacteria or viruses.
Herpes simplex virus, which causes cold sores and genital herpes.
Mycoplasma pneumoniae, which causes a type of atypical pneumonia.
Hepatitis A, which affects the liver.
HIV, which weakens the immune system.
SJS can also be triggered by other factors, such as:
Genetic factors, such as certain human leukocyte antigens (HLAs) that increase the risk of developing SJS.
Environmental factors, such as radiation, sunlight, or chemicals.
Immune system disorders, such as lupus or rheumatoid arthritis.
The symptoms of SJS usually begin with flu-like signs, such as:
Fever.
Headache.
Sore throat.
Cough.
Body aches.
Fatigue.
A red or purple rash appears on the skin within a few days, which becomes blistered as it spreads. The blisters can affect any part of the body, but they are more common on the face, trunk, and limbs. The blisters can also affect the mucous membranes, causing painful sores in the mouth, nose, eyes, genitals, and anus. Symptoms include swelling, inflammation, and tenderness of the skin and mucous membranes.
Eventually, the top layer of the skin, the epidermis, will die and peel off, exposing the underlying layer, the dermis. This can lead to extensive skin loss, dehydration, infection, and shock. Scarring, pigmentation changes, and hair loss may also result.
SJS can also affect other organs and systems, such as:
The eyes, causing dryness, irritation, inflammation, ulcers, scarring, and vision loss.
The lungs, causing breathing difficulties, pneumonia, and respiratory failure.
The kidneys, causing reduced urine output, blood in the urine, and kidney failure.
The liver, causing jaundice, elevated liver enzymes, and liver failure.
The gastrointestinal tract, causing nausea, vomiting, diarrhea, bleeding, and perforation.
The blood, causing anemia, low platelets, and sepsis.
An individual's diagnosis of SJS depends on a number of clinical features, such as the history of medication use or infection, the appearance of the rash and blisters, and the extent of skin involvement. As a way to confirm the diagnosis, a skin biopsy is performed, in which a small sample of the affected skin is taken and examined under a microscope.
There are other tests that can be performed to assess the condition and its complications, including:
Blood tests, to check for infection, inflammation, electrolyte imbalance, organ function, and blood count.
Urine tests, to check for kidney function and infection.
Chest X-ray, to check for lung involvement and pneumonia.
Eye examination, to check for eye damage and vision loss.
The treatment of SJS aims to:
Stop the cause, by identifying and discontinuing the medication or treating the infection that triggered the condition.
By applying moist dressings, creams, and ointments, you can protect and heal the affected skin and mucous membranes. To prevent infection and promote healing, the skin may also need to be debrided, which means removing dead tissue. The damaged areas may need to be covered with healthy skin grafted from another part of the body or from a donor in severe cases.
Dehydration and malnutrition can be prevented and treated with intravenous fluids, electrolytes, and nutrition. In addition to painkillers, sedatives, and anti-inflammatory drugs, antibiotics may also be given to prevent or treat infection. Other medications may be used to treat specific organs or systems, including eye drops, corticosteroids, immunoglobulins, or antivirals.
Follow-up and monitoring of vital signs, blood tests, urine tests, and wound healing are necessary. Depending on the severity of the condition, the patient may need to stay in the hospital for several weeks or months. Long-term rehabilitation and care may also require the patient to see dermatologists, ophthalmologists, and other specialists.
The prevention of SJS involves:
Especially if you have a history of SJS or an allergy to medications that can cause SJS, avoid the medications that cause SJS. Whenever you take any medication, including over-the-counter and herbal products, you should always inform your doctor and pharmacist about your medication allergies and reactions. You should also read the labels and warnings of any medication you take. You should stop taking the medication and seek medical attention immediately if you develop any signs of an allergic reaction, such as rashes, itching, or swelling.
By practicing good hygiene, getting vaccinated, and seeking prompt treatment for any infections you may have, you can reduce your risk of SJS. It is also recommended to avoid contact with people who have infections, especially if you have a weakened immune system.
If you have a family history of SJS or a similar condition, you might consider getting tested for these genes. Being aware of your genetic risk factors, such as certain HLAs that increase your risk of developing SJS. You may also consult your doctor about the benefits and risks of taking certain medications that are associated with SJS, and whether alternative options are available.
