Sydney, Nov 11: Researchers from Australia and Germany have for the first time cured patients suffering from toxic epidermal necrolysis (TEN), a deadly skin disease, said a news release on Monday.
An international collaboration, including researchers from the Walter and Eliza Hall Institute of Medical Research (WEHI) in Melbourne and the Max Planck Institute of Biochemistry in Germany, has developed the first ever cure for TEN in a breakthrough study published in Nature, WEHI said in a news release on Monday.
Also known as Lyell's syndrome, TEN is a rare skin disease that causes widespread blistering and detachment of the skin and can lead to dehydration, sepsis, pneumonia and organ failure, Xinhua news agency reported. The potentially deadly condition is triggered by a severe adverse reaction to common medications and has a mortality rate of approximately 30 per cent.
The new study identified a hyperactivation of the JAK-STAT signaling pathway a chain of interactions between proteins in a cell that is involved in processes such as immunity, cell death and tumour formation as a driver of TEN. By using JAK inhibitors an existing class of drugs used to treat inflammatory diseases they were able to treat patients with TEN."Finding a cure for lethal diseases like this is the holy grail of medical research. I am beyond proud of this incredible research collaboration that has already helped to save the lives of multiple patients," Holly Anderton, an author of the study from WEHI, said.
"All seven people treated with this therapy in our study experienced rapid improvement and a full recovery, in staggering results that have likely unlocked a cure for the condition." Researchers said they are hopeful the findings will pave the way for a clinical trial aimed at the approval of JAK inhibitors as a cure for TEN.