Toxic Epidermal Necrolysis or TEN is an autoimmune disease. It is a more extreme form of Stevens Johnson Syndrome or SJS. In these diseases, the immune system goes haywire, and attacks its own tissue as it would a foreign bacterium or virus. In the case of SJS and TEN, the body attacks its own skin. In some cases, the reaction also includes the mucous membranes like the inside of the nose and mouth. Some cases also may involve the eyes and internal organs. In these diseases, the symptoms are caused by the body attacking itself.

What Are the Symptoms of TEN?

Most cases of TEN start out as SJS. During SJS, the patient first suffers generalized flu-like symptoms such as fever, chills and muscle aches. However, this condition rapidly progresses to a painful rash. The SJS rash includes blisters, sores and ulcers as the body attacks the skin. The most serious cases of SJS progress to TEN.

If SJS progresses to TEN, the symptoms escalate. Patches of the skin die and slough off, leaving the patient with burn-like injuries. These injures leave the patient at risk for many of the same complications seen in burn victims. For example, the wounds of TEN are susceptible to secondary infection, and leave the patient at high risk for dehydration and hypothermia. In both SJS and TEN, the damage can also spread to the internal organs and eyes. In most cases, hospital intensive care units bring TEN patients to their burn wards since the concerns are so similar. Patients may die from TEN complications, damage to the respiratory tract and even organ failure.

Even when patients survive, they can be left with serious complications. Permanent, disfiguring and disabling scars can cover their body. If the reaction spread to the eyes, patients may suffer from permanent blindness.

What Causes TEN?

Many questions remain about TEN. The vast majority of cases appear to occur as a reaction to medication, while very rarely infections and even cancers have been implicated. However, many of the implicated drugs appear to only have this effect on susceptible individuals. Researchers have found that certain genes make people more likely to develop SJS and TEN after taking certain drugs. However, there are numerous instances of people with the genes not getting TEN after using high-risk medication, as well as numerous cases of people getting TEN who lack the high-risk gene. This means that the genetics and etiology of TEN is probably very complex. Still, in most cases when a patient develops TEN, immediate cessation of the medication is ordered. In fact, this is one of the few “treatment” options available.

Drugs that have been allegedly linked to SJS and TEN include:

• Penicillin
• Azithromycin (Zithromax/Z-Paks)
• Lamotrigine (Lamictal)
• Diclofenac
• Acetaminophen (the active ingredient in Tylenol)