Steven-Johnson syndrome
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Stevens-Johnson syndrome (SJS) is a severe skin disorder that typically necessitates hospitalization. This condition causes rashes, blisters, and peeling skin, also affecting mucus membranes like the eyes, mouth, and genitalia. It is often triggered by a medication reaction, and healing process may take weeks or months.
Some people consider SJS and toxic epidermal necrolysis (TEN) as different diseases, while others view them as the same disease with SJS being less severe. In SJS, less than 10% of the skin peels, while in TEN, over 30% of the skin is affected. However, both conditions can be life-threatening.
SJS is a severe condition that necessitates immediate medical attention and typically requires admittance to a hospital for proper treatment. If the condition was caused by a medication, permanently avoiding that drug and similar ones may help. Treatment for SJS usually focuses on treating the underlying cause, tending to wounds, managing pain, and reducing complications as the skin heals.
Symptoms
One to three days before a rash develops, early signs of Stevens-Johnson syndrome may include fever, a sore mouth and throat, fatigue, and burning eyes.
Common symptoms of Stevens-Johnson syndrome include:
- Pain on the skin
- Painful throat and mouth
- Cough
- Spreading red or purple rashes on the body
- Ulcers and blisters on the skin, as well as on the mucous membranes of the eyes, mouth, throat, genitalia, and anus
- Skin shedding that occurs days after blister formation
- Painful urination as a result of mucus membrane blisters
If any symptoms persist, seeking prompt medical attention is advisable for accurate diagnosis and treatment. Medications can potentially trigger this condition, even after discontinuation, with symptoms possibly resurfacing up to two weeks later. Recurrence is possible, typically with heightened severity upon subsequent occurrences.
Causes
Stevens-Johnson syndrome is an uncommon and unpredictable condition. If a drug triggers it, symptoms appear one to three weeks after starting the medication, beginning with flu-like symptoms, and followed by a rash and skin peeling. In severe cases, hair and nails may also be lost.
Stevens-Johnson syndrome can be caused by an allergic reaction to medication, infections like pneumonia and herpes, vaccinations, graft-versus-host disease, or sometimes no known cause.
Medications associated with Stevens-Johnson Syndrome encompass anti-gout drugs such as allopurinol, anticonvulsants, antipsychotics, sulfonamide antibiotics, nevirapine, and pain relievers like acetaminophen, ibuprofen, and naproxen sodium.
Risk factors
Factors that increases the risk of SJS include:
- Having SJS from a medication before, and taking that drug again
- Having a close relative or immediate family member with SJS
- Having certain genetic factors, especially if taking drugs for seizures, gout, or mental illness
- Having HIV, which increases the risk for about 100 times
- A weakened immune system, due to organ transplants, HIV/AIDS, or autoimmune diseases, raises the risk
- Having cancer, especially blood cancer
Diagnosis
Diagnosing SJS entails a thorough inquiry into the individual’s health background, encompassing current and recently ceased medications, along with a comprehensive physical assessment. The examination focuses on inspecting the afflicted skin and mucus membranes. Factors such as the intensity of pain, rapidity of skin involvement, and the scope of affected skin are carefully evaluated during the diagnostic process.
Tests that may be required include:
- Blood tests: Presence of infection or other potential causes may be identified in this test.
- X-ray: This can help examine for conditions such as pneumonia that may be causing the symptoms.
- Culture: This involves collecting a sample of skin, tissue, or fluid for laboratory testing (culture) in order to rule out an infection.
- Skin biopsy: Skin sample is extracted for lab analysis to rule out potential causes or confirm a diagnosis.
Treatment
Management of Stevens-Johnson syndrome encompasses multiple strategies, including discontinuation of the causative medication, administering supportive care, and adhering to prescribed medications. Hospitalization, which may include intensive care or burn unit support, could be warranted. Collaborative efforts involving specialist teams from dermatology and ophthalmology may be required, particularly if ocular complications arise.
Treatments for SJS:
- Avoiding triggering medications: Healthcare providers might advise stopping all nonessential medications to prevent further harm, especially in people on multiple drugs. This is the initial and crucial treatment for Stevens-Johnson syndrome.
- Medications: Antibiotics may be used to control infections, while pain relief medications are provided for comfort. Other medications include:
- Topical steroids: These can lessen inflammation in the mucous membranes and eyes.
- Oral and systemic drugs: Such as IV immunoglobulin and corticosteroids. Research indicates that etanercept and cyclosporine are effective medications for this illness.
- Supportive care:
- Replenishing electrolytes and nutrition: Administering high-calorie nutrition, possibly via tube-feeding, and replenishing electrolytes through intravenous fluids can be part of the treatment. Replacing lost fluids is an important element of treatment since skin loss can cause significant fluid loss from the body.
- Wound care: Applying non-adhesive dressings on the affected skin can aid the healing. This include using medicinal dressing or petroleum jelly to treat the afflicted areas after carefully removing any dead skin. Cool, moist compresses may be used to ease blisters while they heal.
- Eye care: You may also require care from an ophthalmologist.
If the reason for SJS is removed and skin reaction halted, a new skin might start growing within a few days. Severe cases of SJS could take several months for complete recovery.
