Toxic epidermal necrolysis (TEN)
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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).
Diagnosis
Healthcare providers typically diagnose toxic epidermal necrolysis (TEN) by evaluating your skin and symptoms. When individuals with SJS exhibit a severe illness impacting more than 30% of their bodies, TEN is identified. Occasionally, confirmation of the diagnosis may necessitate a skin biopsy. A pathologist, a healthcare professional specializing in examining human tissues, analyzes a sample of your skin under a microscope to confirm the diagnosis.
Treatment
It will be necessary for you to stop taking the medication if your healthcare provider considers it may have contributed to your TEN. Afterwards, you will probably be admitted to a hospital and treated there, perhaps in an ICU or burn center. It may take several months for everything to recover completely.
The primary goal of TEN treatment is to maximize your comfort level as your skin heals. During your stay in the hospital, you will get supportive care. It could consist of:
- Fluid replacement and nutrition: Replacing fluids and electrolytes is important since skin loss can cause the body to lose fluids. A nasal tube (nasogastric tube) that is inserted in the nose to the stomach may be used to provide nutrition and fluids.
- Wound care: The healthcare provider may gently wash the affected area and apply bandages that are infused with medicine or petroleum jelly (Vaseline). Additionally, your healthcare provider keeps an eye out for infections and may administers antibiotics as necessary.
- Breathing support: To assess your airway and maintain its clarity, you may require tests and procedures. You may require artificial breathing assistance (ventilation) or intubation if your condition is severe.
- Pain management: Painkillers will be administered to you to ease your pain. You may be prescribed mouthwash that contains lidocaine or another numbing ingredient if you have oral pain.
- Eye care: Applying preservative-free artificial tears at least four times a day may help with mild problems with the eyes. To reduce inflammation in the eyes, corticosteroid-containing eye drops may be applied.
- Other treatments: For the treatment of TEN, a patient may also be prescribed systemic medicines:
- Immune globulin: Immunoglobulin is administered intravenously (IV). It is a sterile fluid derived from donor plasma, which is the liquid portion of blood. It has components or antibodies that support your body’s defense against infections.
- Immunosuppressants: When the immune system isn’t functioning properly, these medications weaken it. They prevent tissue-attacking cells in your body.
- Plasmapheresis: By removing blood from your body, this process also extracts plasma from blood cells. Materials that trigger a negative immunological reaction are extracted from the plasma by a machine. The “clean” blood or a plasma replacement is then given back to your body by the machine.
