Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: Recognizing Medication-Related Emergencies

What You Need to Know About Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) aren’t just rare skin rashes. They’re life-threatening emergencies triggered by medications, and they can turn deadly within days if missed. The first sign? A fever, sore throat, or flu-like feeling that doesn’t go away. Then comes the rash-red, painful, spreading fast. Blisters form. Skin starts peeling off. Eyes, mouth, and genitals burn. This isn’t an allergic reaction you can treat with antihistamines. This is a full-thickness skin death. And it demands immediate hospitalization.

Doctors see SJS and TEN as points on the same spectrum. SJS affects less than 10% of your body surface. TEN? More than 30%. Between 10% and 30%, it’s called SJS/TEN overlap. The bigger the area of skin lost, the higher the chance of death. Mortality jumps from 5% in mild SJS to over 30% in severe TEN. Most deaths come from sepsis, organ failure, or internal bleeding-not the rash itself. But the rash is the warning sign.

Which Medications Cause These Reactions?

It’s not random. Certain drugs carry a known, documented risk. The biggest culprits? Anticonvulsants like lamotrigine, carbamazepine, phenytoin, and phenobarbital. Antibiotics like sulfamethoxazole (part of Bactrim) and penicillin derivatives. NSAIDs like piroxicam and meloxicam. And allopurinol, the common gout drug. These aren’t obscure drugs-they’re prescribed millions of times a year.

Here’s the catch: you don’t have to be on the drug for long. Most reactions start within the first 8 weeks. Sometimes, they appear even after you’ve stopped taking it-up to two weeks later. That’s why people think they’re safe once they’ve switched medications. They’re not. And if you’ve had SJS or TEN from one drug, you’re at high risk if you take another drug in the same class. Cross-reactivity is real. If lamotrigine caused it, avoid carbamazepine and phenytoin. If sulfamethoxazole triggered it, steer clear of other sulfonamides.

Who’s at Higher Risk?

It’s not just about the drug. Genetics and health history matter. People with HIV or those on chemotherapy have weaker immune systems and face higher risk. So do those who’ve had a previous rash from epilepsy meds-even if it was mild. Taking sodium valproate with lamotrigine increases the chance of a bad reaction. And if someone in your family had SJS or TEN, your risk may be higher. Certain genes, like HLA-B*15:02 in people of Asian descent, are linked to carbamazepine-induced SJS. That’s why some doctors test for it before prescribing.

Children are more likely to get SJS than adults, but adults are more likely to die from it. Why? Because adults often have other health problems-diabetes, kidney disease, heart conditions-that make recovery harder. And older adults are more likely to be on multiple medications, increasing the chance of a dangerous interaction.

What Does It Look Like? Recognizing the Early Signs

Don’t wait for skin to peel. By then, it’s too late. Watch for these early signs:

• High fever (over 101°F)
• Sore throat, burning eyes, or mouth ulcers
• Flu-like fatigue that doesn’t improve
• A red or purplish rash that spreads quickly
• Blisters on skin, lips, or inside the mouth
• Painful skin that feels like it’s burning

The rash doesn’t itch like hives. It hurts. It feels like a bad sunburn that’s turning into raw meat. Blisters burst easily. Skin sloughs off in sheets. Mucous membranes-eyes, mouth, throat, genitals-are almost always involved. If you have blisters in your mouth and a rash on your chest? That’s not a virus. That’s SJS.

Doctors confirm it with a skin biopsy. Under the microscope, they see full-thickness epidermal necrosis-dead skin layers peeling away from the body. No inflammation. No infection. Just death of the outer skin layer. That’s the hallmark.

What Happens in the Hospital?

There’s no magic pill. Treatment is supportive care-like treating a major burn. You’ll be moved to a burn unit or ICU. IV fluids, pain control, and wound care are critical. Your skin is exposed. Infection is the biggest threat. Antibiotics aren’t used unless there’s clear infection-they don’t prevent it. Steroids and immunoglobulins are sometimes tried, but studies show mixed results. The most important step? Stop the drug immediately. No exceptions.

Eye care is urgent. The cornea can scar permanently. You’ll need daily eye drops, sometimes surgery. Mouth sores make eating impossible. Nutrition often comes through a feeding tube. Genital ulcers can cause scarring and narrowing-leading to lifelong problems with urination or intercourse. Nail loss is common. Hair may thin out. Recovery takes weeks to months. And even after healing, complications can last years.

Long-Term Damage: It Doesn’t End When You Leave the Hospital

Survivors aren’t just lucky-they’re burdened. Up to half of people who survive SJS/TEN develop serious eye problems: dry eyes, light sensitivity, scarring, even blindness. Oral complications mean chronic dry mouth, gum disease, and difficulty swallowing. Some develop esophageal strictures-narrowing that makes eating solid food painful. Women may face vaginal stenosis. Men may get phimosis. Nails grow back slowly, if at all. Skin can be permanently scarred or discolored.

These aren’t rare side effects. They’re expected outcomes. That’s why survivors need long-term follow-up: dermatologists, ophthalmologists, dentists, and gynecologists or urologists. One study found that 30-50% of survivors had lasting vision damage. That’s not a small number. That’s nearly half.

How to Prevent It

Prevention starts with awareness. If you’re prescribed lamotrigine, carbamazepine, allopurinol, or sulfamethoxazole, know the signs. Don’t ignore a rash. Don’t wait to see if it gets better. If you get a fever and rash within the first 8 weeks of starting a new drug, go to the ER. Don’t call your doctor. Don’t wait for an appointment. Go now.

For lamotrigine users: never restart the drug after stopping it-even for a few days-without slowly increasing the dose again. Rapid dose increases are a known trigger. Avoid new foods or supplements in the first 3 months. They can confuse symptoms. If you’ve had a rash from any epilepsy drug before, tell your doctor before taking any new one.

Genetic testing isn’t routine, but if you’re of Asian descent and your doctor plans to prescribe carbamazepine, ask about HLA-B*15:02 screening. It’s simple, cheap, and can prevent death.

What to Do If You Suspect SJS or TEN

Stop the medication. Immediately. Then get to the nearest emergency room. Tell them: “I think I have Stevens-Johnson Syndrome.” Don’t say “I have a rash.” Say the name. It matters. Emergency staff need to recognize this fast. The sooner you’re in a burn unit, the better your chance of survival.

Bring a list of all your medications-prescription, over-the-counter, supplements. Even one pill you took a week ago could be the trigger. Don’t assume it’s harmless. Write down when you started each drug and when symptoms began. That timeline helps doctors pinpoint the cause.

And if you survive? Never take the drug again. Or any similar one. Keep a medical alert bracelet. Tell every doctor you see. SJS/TEN is a lifelong warning sign.

Final Reality Check

Yes, SJS and TEN are rare. The risk is less than five cases per million users per week. But rarity doesn’t mean safety. It means unpredictability. One person out of a million might react. But if it’s you? It’s 100%. And the consequences are devastating. There’s no second chance. No do-over. No waiting to see if it clears up.

Medications save lives. But they can also kill-quietly, fast, and without warning. The key isn’t avoiding all drugs. It’s knowing which ones carry this hidden risk. Recognizing the early signs. Acting immediately. And never underestimating a rash that won’t go away.