Stevens-Johnson Syndrome (SJS) may sound exotic to most people, but the fatal disease, caused by a serious adverse reaction to medication, affects a substantial number of people each year and accounts for about half of the cases handled by the Taiwan Drug Relief Foundation, Academia Sinica said yesterday.
Help may be on the way, however, with the discovery by Chung Wen-hung (鍾文宏), a dermatologist at Chang Gung Memorial Hospital in Taipei, that the severe and progressive disease is strongly associated with granulysin.
Granulysin, a substance that exists naturally in the body, can attack foreign substances or organisms, Academia Sinica said, adding that treatment research was under way.
The discovery will be published in this week's edition of the biomedical research journal Nature Medicine, it said.
“People suffering from SJS, or its more severe form — toxic epidermal necrolysis — are characterized by general blistering of the skin as well as skin lesions,” Chung said, adding that as skin cells die, they cause the epidermis to separate from the dermis.
In his study, Chung found granulysin, a protein that exists naturally in the body's immune system, attacking an SJS sufferer's skin and mucosa cells.
“By secreting granulysin when immune cells try to defend against invading organisms or substances, the immune cells in turn also kill the bystanding host cells, causing irreversible cell and tissue damage,” Chung said.
The finding could provide invaluable insight into the mechanism of other immune cell-mediated diseases, Chung's thesis adviser, Chen Yuan-tsong (陳垣崇), said.
Chung had just been awarded a doctorate degree from the Taiwan International Graduate Program, which is cosponsored by Academia Sinica and National Yang Ming University.
“Granulysin may be used as a therapeutic target for further development of an effective treatment for some of the very severe immune-mediated disorders which currently have no satisfactory treatment,” Chen said.
This was the second time for the 37-year-old Chung — who was educated and trained in Taiwan and is described by Academia Sinica as a “home-grown Ph.D.” — to have a paper published in the journal.
Chen lauded his protege's achievements, saying: “Obviously we are very proud of him.”
Chung's research also appeared in Nature Medicine in 2004, when he discovered that a specific human leukocyte antigen is strongly associated with SJS patients taking Carbamazepine, Chen said.
Carbamazepine is a drug commonly used to treat seizures.
Chung's discovery prompted Taiwan's Department of Health and the US Food and Drug Administration to recommend genetic screening before doctors prescribe the drug, Chen said.
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