Summary about Disease
Stevens-Johnson Syndrome (SJS) is a rare, serious disorder of the skin and mucous membranes. It's usually a reaction to medication or an infection. SJS causes a painful rash, blisters, and detachment of the top layer of skin. It's considered a medical emergency requiring hospitalization.
Symptoms
Early symptoms can resemble the flu, such as:
Fever
Sore throat
Fatigue
Cough
Burning eyes As the condition progresses, other symptoms develop:
Unexplained widespread skin pain
A red or purplish rash that spreads
Blisters on the skin and mucous membranes (mouth, nose, eyes, genitals)
Shedding of skin
Causes
The most common cause of SJS is a reaction to medications, especially:
Anti-seizure drugs (anticonvulsants)
Antibiotics (especially sulfonamides)
Pain relievers (NSAIDs like ibuprofen and naproxen)
Allopurinol (used for gout) Infections can also cause SJS, including:
Herpes simplex virus
Pneumonia
HIV Sometimes, the cause of SJS cannot be determined (idiopathic).
Medicine Used
Treatment focuses on managing symptoms and preventing complications. There is no specific cure for SJS. Medications used may include:
Pain relievers: To manage pain and discomfort.
Antihistamines: To relieve itching.
Topical antibiotics/antiseptics: To prevent infection of open skin areas.
Intravenous immunoglobulin (IVIG): May help stop the progression of the disease in some cases.
Corticosteroids: Their use is controversial and may be considered in specific situations.
Immunosuppressants: Medications like cyclosporine or TNF inhibitors are sometimes used in severe cases.
Is Communicable
SJS itself is not communicable. It is a reaction within an individual's body, not an infection that can be spread to others. However, if an infection (like herpes) is the underlying cause, that infection could be communicable.
Precautions
Medication awareness: Be aware of potential side effects of medications you are taking, especially if you have a history of drug allergies or reactions.
Prompt medical attention: Seek immediate medical care if you develop a rash, blisters, or flu-like symptoms after starting a new medication.
Allergy documentation: Keep a detailed record of all drug allergies and reactions and share it with your healthcare providers.
Genetic testing: Some individuals may have genetic predispositions that increase their risk of developing SJS with certain medications. Genetic testing can sometimes help identify these risks.
How long does an outbreak last?
The acute phase of SJS typically lasts from 2 to 4 weeks. Full recovery can take several weeks to months, and some individuals may experience long-term complications.
How is it diagnosed?
Diagnosis of SJS is based on:
Physical examination: Assessing the skin and mucous membrane lesions.
Medical history: Reviewing medications and potential exposures.
Skin biopsy: A small sample of skin is examined under a microscope to confirm the diagnosis and rule out other conditions.
Blood tests: To evaluate overall health and rule out infections.
Timeline of Symptoms
Early Phase (1-3 days): Flu-like symptoms (fever, sore throat, fatigue, cough, burning eyes).
Active Phase (days 4-10): Rash develops, spreading rapidly. Blisters appear on skin and mucous membranes. Skin pain becomes significant.
Peak Severity (days 10-14): Skin shedding occurs (often described as "sloughing"). Risk of complications (infection, dehydration) is highest.
Recovery Phase (weeks-months): Skin begins to heal. Pain and inflammation gradually decrease. Long-term complications may become apparent.
Important Considerations
Severity: SJS can range from mild to life-threatening. The severity is often assessed using a scoring system called SCORTEN (Score of Toxic Epidermal Necrolysis).
Complications: Potential complications include skin infection (cellulitis, sepsis), dehydration, eye damage (scarring, vision loss), lung problems, and multiple organ failure.
Psychological impact: SJS can be emotionally traumatic. Support groups and counseling may be helpful.
Recurrence: While rare, SJS can recur, especially if the offending medication is taken again.
Overlap with TEN: SJS and Toxic Epidermal Necrolysis (TEN) are considered variations of the same disease. TEN is a more severe form with more extensive skin detachment (greater than 30% of body surface area). SJS/TEN overlap is between 10-30% detachment.