A rare and deadly skin disease may finally have a treatment! Toxic epidermal necrosis (TEN), also called Lyell syndrome, occurs unpredictably. This is a toxidermia, resulting from side skin effects related to taking medications. The prognosis for this disease remains guarded, particularly due to numerous complications. In addition, its treatment is long, not very effective, and similar to that used for burn victims. However, a new study brings hope since it indicates that it offers a treatment offering a complete cure for this pathology. The scientists published their results in the journal Nature.
The toxic epidermal necrosis, a rare and fatal skin disease
Toxic epidermal necrosis (TEN) is an unpredictable drug. It is a rare and fatal disease of skin which strongly affects people's body affected. In fact, the latter see their skin and mucous membranes detach.
This is a toxidermie fortunately very rare. There is approximately 1 new case per year per million inhabitants worldwide. Its main characteristic is the brutal destruction of the superficial layer of the skin and mucous membranes.
People complain of the appearance of skin lesions, which peel off at the slightest friction. The dermis, the middle layer of the skin, is then laid bare. It becomes bright red, itchy and painful.
The disease is also accompanied by visceral damage, particularly at the respiratory and digestive levels.
This rare and fatal skin disease is often triggered following when taking certain medications. Sulphonamides, antiepileptic medications, nonsteroidal anti-inflammatories (of the oxicam type) or even antiretroviral medications are most often incriminated.
The Pathology is mainly observed in adults, but can occur at any age, in both sexes. Treatment is essentially symptomatic and is similar to that applied to serious burn victims.
Unfortunately, the disease is often accompanied by complications, sometimes fatal. Fluid and hydro-electrolyte losses are often significant and lead to acute renal failure. The skin no longer acts as a natural barrier, patients contract serious systemic infections and present thermoregulation disorders.
In extensive forms, the prognosis is serious. Mortality occurs in 20 to 25% of cases. Age (greater than 40 years) and the percentage of detached body surface (greater than 10%) are important mortality criteria. People who survive very often suffer after-effects (in more than 80% of cases). The ocular after-effects are the most worrying and require the use of ophthalmic devices.
Despite its rarity, its seriousness and its impact clearly demonstrate the importance of finding an effective treatment, allowing a complete cure.
Toxic epidermal necrolysis, a rare and fatal skin disease, is mainly linked to the use of certain medications.
Credits: Shutterstock/Tamakhin Mykhailo
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The hope of a complete cure thanks to an innovative treatment
An international team of researchers wanted to discover a remedy. They mainly studied a certain class of drugs, JAK inhibitors. These appear to work by suppressing an overactive immune pathway.
This is particularly interesting since this rare and deadly skin disease is an immune reaction to certain medications. Finding alternatives to block this reaction therefore seems to be a good avenue.
Scientists have notably discovered the importance of this signaling pathway using skin samples from patients suffering from toxic epidermal necrosis. The goal was to discover which individual cells caused this deadly disease. They thus identified 6 proteins playing a role in this immune pathway. Proteins that are in excessive quantities in people with NET.
This immune pathway, called JAK/STAT, is the main factor in skin inflammation. It is also the cause of skin lesions and detachment of the epidermis.
The researchers then administered an inhibitor of this JAK/STAT pathway to mice suffering from NET. They noticed a very clear improvement in skin infections.
They continued their test further by applying it directly to sick humans. The compelling preclinical data and urgent need for treatment allowed researchers to treat 7 patients.
All responded very well and experienced rapid improvement and complete recovery. This is particularly the case for one of the patients. This is a 59-year-old man who developed toxic epidermal necrosis following treatment for lung cancer. The disease affected 35% of his body and his predicted mortality risk was 60%.
Thanks to the inhibitor, his infection stopped progressing. After 16 days, he was almost completely cured!
This inhibitor treatment is therefore a real hope in the fight against this rare and fatal skin disease. It could make a big difference in the quality of care and patient care.
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With an unwavering passion for local news, Christopher leads our editorial team with integrity and dedication. With over 20 years’ experience, he is the backbone of Wouldsayso, ensuring that we stay true to our mission to inform.
