Toxic epidermal necrolysis (TEN)

Overview

Toxic epidermal necrolysis (TEN) represents a critical, potentially fatal skin disorder often triggered by medications, acting as an intensified variant of Stevens-Johnson syndrome (SJS). The distinction into TEN is made when the affliction expands to over 30% of the skin’s surface, alongside considerable harm to the body’s mucous membranes. This condition does not discriminate by age, posing a threat to individuals across all age groups. Typically, management of TEN necessitates hospitalization, where a multifaceted approach to care is employed. This includes pain management, meticulous wound care, and ensuring adequate fluid intake, all pivotal for the skin’s recovery process. The healing timeline can span from weeks to months, contingent upon the severity of the case.

If a specific medication is identified as the trigger for TEN, it is essential for the patient to permanently avoid that drug and any similar substances. This precaution is critical to prevent the recurrence of this severe reaction and to protect the patient’s health and safety.

Symptoms

Symptoms of Toxic Epidermal Necrolysis (TEN) can appear a few weeks after initiating a new medication, initially presenting as symptoms similar to the flu, such as:

• Coughing.
• Muscle pain.
• Headaches.
• High temperature and shivers.
• Eye redness and swelling.

As TEN progresses, it leads to a severe skin rash that becomes excruciating, eventually evolving into extensive blistering and skin peeling. This results in the formation of erosions, which are agonizing open sores resembling burns, as the skin layers detach.

These erosions typically begin on the face and chest and may extend to other body parts, including:

• The eyes, affecting the conjunctiva (the inside part of the eyelids) and the cornea (the eye’s outer lens).
• Mucosal surfaces, such as the interior of the mouth, nose, throat, and respiratory tract.
• The urinary system, anus, and genital area.

These erosions can severely hamper basic functions, making it difficult and painful to eat, swallow, breathe, see, and use the bathroom.

Causes

A medication-related skin reaction is typically the cause of SJS/TEN. One to four weeks after you begin taking a new medication, the symptoms will begin to manifest. The following medication may cause TEN:

• Allopurinol (medication for kidney stones and gout).
• Anticonvulsants (medication to treat seizures).
• HIV treatment anti-retroviral (particularly non-retroviral therapy inhibitors, such as nevirapine, efavirenz, or etravirine).
• Oxicams, a class of NSAIDs, or non-steroidal anti-inflammatory medicines.
• Sulfonamides (a type of antibiotic).
• Vaccinations.

In some cases, TEN’s cause is unknown.

Risk factors

The risk of SJS/TEN is increased by the following factors:

• History of SJS/TEN: Recurrence risk increases if you use the medication that caused your medication-related type of this condition. Your chances of getting SJS/TEN may increase if a parent or sibling, or other first-degree relative, has had the condition.
• Genetic factors: A higher risk of SJS/TEN is associated with specific genetic variations, particularly if you also take medication for gout, seizures, or mental health issues.
• HIV infection: The incidence of SJS/TEN is approximately 100 times higher in HIV-positive individuals than in the general population.
• Weak immune system: HIV/AIDS, autoimmune disorders, and organ transplants can all have an effect on the immune system.
• Cancer: Individuals who have cancer are more likely to develop SJS/TEN, particularly blood tumors (hematologic malignancies).