Overview
Stevens-Johnson syndrome (SJS) is a rare, serious disorder of the skin and mucous membranes. It usually occurs as a reaction to medication and starts with flu-like symptoms, followed by a painful rash that spreads and blisters. The top layer of affected skin dies, sheds, and begins to heal after several days. SJS is a medical emergency that requires hospitalization and can take weeks to months to recover. If caused by medication, it is necessary to permanently avoid that drug and others like it.
Understanding the disease
Symptoms
The symptoms of Stevens-Johnson syndrome include:
- Fever
- A sore mouth and throat
- Fatigue
- Burning eyes
- Unexplained widespread skin pain
- A red or purple rash that spreads
- Blisters on your skin and the mucous membranes of the mouth, nose, eyes, and genitals
- Shedding of skin within days after blisters form These symptoms require immediate medical attention, and the condition should be diagnosed by a healthcare professional.
Risk Factors
The risk factors for developing Stevens-Johnson syndrome include:
- A history of Stevens-Johnson syndrome: If you've had a medication-related form of this condition, you are at risk of a recurrence if you use that drug again.
- A family history of Stevens-Johnson syndrome: If an immediate blood relative has had Stevens-Johnson syndrome, you may be at increased risk of getting it too.
- Genetic factors: Having certain genetic variations puts you at increased risk of Stevens-Johnson syndrome, especially if you're also taking drugs for seizures, gout, or mental illness.
- Cancer: People with cancer, particularly blood cancer, are at increased risk of Stevens-Johnson syndrome.
- HIV infection: Among people with HIV, the incidence of Stevens-Johnson syndrome is about 100 times greater than among the general population.
- Weakened immune system: The immune system can be affected by an organ transplant, HIV/AIDS, and autoimmune diseases.
- Use of specific medications: Certain medications, such as anti-gout medications, medications to treat seizures and mental illness (anticonvulsants and antipsychotics), antibacterial sulfonamides (including sulfasalazine), nevirapine (Viramune, Viramune XR), pain relievers like acetaminophen, ibuprofen, and naproxen sodium, and others, can increase the risk of developing Stevens-Johnson syndrome. It's important to avoid the medication that triggered the condition if you have a history of Stevens-Johnson syndrome, and to consult with a healthcare provider about alternative medication options.
Development
Stevens-Johnson syndrome (SJS) and its more severe form, toxic epidermal necrolysis (TEN), develop due to a reaction to medication, infection, or both. The condition typically begins with flu-like symptoms, followed by a painful rash that spreads and blisters. The top layer of affected skin dies, sheds, and begins to heal after several days. SJS usually requires hospitalization, with treatment focusing on removing the cause, caring for wounds, controlling pain, and minimizing complications as skin regrows. Recovery can take weeks to months, and permanent skin damage may occur, including bumps, unusual coloring, and scars. Hair loss and poor nail growth may also be permanent.
Assessment and Diagnosis
Triage
You should see a doctor immediately if Stevens-Johnson syndrome is suspected. This condition requires emergency medical attention. If you experience early signs such as fever, sore mouth and throat, fatigue, and burning eyes, or if you develop a rash, blisters, and skin shedding, seek medical help immediately. It is crucial to get medical care as soon as possible to manage the condition effectively and prevent potential complications.
Diagnosis
A diagnosis of Stevens-Johnson syndrome is typically made based on a review of the patient's medical history, a physical examination, and laboratory tests. Health care providers often can identify the condition based on the patient's medical history, including a review of their current and recently stopped medications. A skin biopsy is also performed to confirm the diagnosis and rule out other possible causes. Laboratory tests, such as blood tests, cultures, and imaging, may be used to confirm infection or other possible causes.
Management and Treatment
Complications
Complications of Stevens-Johnson syndrome include dehydration due to fluid loss from skin shedding and sores in the mouth and throat that make fluid intake difficult. Other potential complications may involve permanent skin damage, hair loss, and nail growth problems. In severe cases, the condition can lead to acute respiratory failure and require emergency medical attention. A recurrence of Stevens-Johnson syndrome is also possible, usually with a more severe outcome, particularly if the triggering medication is reused.
Prevention
While there is no definitive way to prevent Stevens-Johnson syndrome, you can take certain measures to reduce your risk:
- Be aware of medications: Before starting a new medication, consult your healthcare provider and discuss any potential risks or side effects, especially if you have a history of SJS or a weakened immune system.
- Avoid certain medications: If you have had SJS in the past and know that a medication triggered it, avoid that drug and similar ones.
- Get genetic testing: The U.S. Food and Drug Administration recommends screening for the HLA-B*1502 gene variation in people of Asian and South Asian ancestry before starting certain medications.
- Maintain a strong immune system: Keeping your immune system healthy can help reduce your risk of developing SJS. This can be achieved through a balanced diet, regular exercise, and proper sleep.
- Manage underlying health conditions: If you have HIV, cancer, or an autoimmune disease, work closely with your healthcare provider to manage these conditions effectively. Remember that early recognition of the signs and symptoms of SJS can lead to prompt treatment and better outcomes. If you experience flu-like symptoms followed by a painful rash, consult your healthcare provider immediately.
Treatment
The main goal of treatment for Stevens-Johnson syndrome is to remove the cause, care for wounds, manage pain, and minimize complications as the skin regrows. Treatment typically occurs in a hospital setting and may involve the following interventions:
- Medication withdrawal: If the syndrome is caused by a medication, the medication should be discontinued immediately.
- Supportive care: Patients are usually treated with fluid and electrolyte replacement to prevent dehydration and maintain blood pressure. Pain management is also crucial to alleviate discomfort.
- Wound care: Topical and oral antibiotics may be prescribed to prevent infection, and wound care is essential to prevent secondary infections and promote healing.
- Nutritional support: In severe cases, a feeding tube may be required to ensure proper nutrition and hydration.
- Monitoring and management of complications: Close monitoring of vital signs, oxygenation, and kidney and liver function is necessary to manage potential complications such as respiratory failure, septicemia, and organ dysfunction. Note that the treatments mentioned here are general approaches to managing Stevens-Johnson syndrome. Specific treatments may vary depending on the severity of the condition, the underlying cause, and individual patient factors. It's essential to seek immediate medical attention if you suspect you or someone you know is experiencing symptoms of Stevens-Johnson syndrome.