Researchers like Wen-Hung are asking why.

SJS is a life-threatening hypersensitivity condition that causes rashes to form over the body. The rashes blister over, causing the skin to peel off in sheets. SJS also can affect the mucus membranes, causing blisters to form in the mouth and eyes. Death can result in about 10 percent of patients with SJS. If it progresses to its most severe form, toxic epidermal necrolysis, (TEN) the death rate soars to 40 to 50 percent. SJS/TEN is linked to more than 200 medications, most commonly ibuprofen, antibiotics and anti-seizure medication.

A group of Chang Gung researchers were aware that the anticonvulsant carbamazepine was linked to many cases of SJS, but through its own study using Taiwanese patients, researchers discovered a possible connection – “a high correlation between hypersensitivity to carbamazepine and a single allele, human leukocyte antigen B1502,” according to the report. As many as 80 percent of Taiwanese carry the allele. The discovery lead to an FDA warning in the U.S. of the increased risk of SJS/TEN for patients of Asian ancestry with the B1502 allele who take carbamazepine.

Since the discovery, researchers began focusing on developing a test to determine whether a patient has the B1502 allele, and lead researcher Wen-Hung turned his attention on a treatment to halt the progression of SJS/TEN.

SOURCE: Stevens Johnson Syndrome › Taiwanese researchers pinpoint genetic marker for SJS

Chung Wen-hung with Chang Gung Memorial Hospital in Taipei, has discovered that granulysin, a naturally occurring substance in the human body, can attack foreign substances or organisms. This discovery may lead to a therapeutic target for further development of an effective treatment for severe immune-mediated disorders that currently have no satisfactory treatment, according to the report. The report is published in last week’s edition of the biomedical research journal Nature Medicine.

SJS accounts for about half of the cases handled by the Taiwan Drug Relief Foundation. It is a rare and sometimes fatal skin disorder that often is caused by medication. SJS and its more severe form, toxic epidermal necrolysis (TEN), are characterized by painful blistering of the skin and skin lesions.

As the condition evolves and skin cells begin to die, skin can literally slough off. Treatment usually focuses on eliminating the underlying cause, if possible, and controlling the symptoms. Recovery can take several weeks to several months.

Chung’s research also appeared in Nature Medicine four years ago when he discovered a link between carbamazepine, an anti-seizure medicine, and patients with SJS. His discovery prompted the Taiwan Department of Health as well as the U.S. Food and Drug Administration to alert healthcare providers of the possible link and recommend genetic screening for the gene before prescribing the medication.

SOURCE: Stevens Johnson Syndrome › Chinese doctor’s discovery may lead to treatment for SJS

In a recent Information for Healthcare Professionals sheet, the FDA described the increased risk of SJS/TEN with another antiepileptic drug, carbamazepine, in patients of Asian ancestry with the HLA-B*1502 allele. HLA-B is a human gene that provides instruction for making a protein that plays a critical role in the immune system. It is part of a family of genes called the human leukocyte antigen (HLA) complex. This complex helps the immune system distinguish the body’s own proteins from proteins made by viruses or bacteria. Several variations of the HLA-B gene are associated with adverse reactions to certain drugs.

SJS is a disorder of the skin and mucous membranes and often begins with flu-like symptoms followed by inflammation of mucous membranes and a painful red or purplish rash that spreads and blisters. It eventually causes the top layer of skin to die and shed.

Until the FDA completes its review, the agency warns healthcare providers who are considering the use of phenytoin or fosphenytoin for their patients to be aware of the risks and benefits described in the current prescribing information for the drug. Healthcare providers should consider avoiding phenytoin and fosphenytoin as alternatives for carbamazepine in patients who test positive for HLA-B*1502.

SOURCE: Stevens Johnson Syndrome › FDA warns of link between anti-epilepsy drugs and SJS