Stevens-Johnson syndrome presents clinical, legal challenges

February 1, 2011

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) require physicians not only to have effective clinical strategies, but also to recognize the legal and interpersonal consequences that can be associated with these conditions.

Key Points

Toronto - Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) require physicians not only to have effective clinical strategies, but also to recognize the legal and interpersonal consequences that can be associated with these conditions.

From one clinician's perspective, SJS/TEN exists on a continuum that may or may not conform to textbook definitions. According to Neil Shear, M.D., professor and chief of dermatology, University of Toronto Medical School, "At the milder end of this spectrum, people will have tiny lesions and hemorrhagic mucositis in the mouth and inflammation of other mucous membranes." Blisters can develop as SJS progresses, "and the more blisters a patient gets, the more likely he or she is to meet the definition of TEN."

Regarding risk management, Dr. Shear says the stages of the SJS/TEN life cycle include identifying what caused the drug reaction, treating its acute manifestations and addressing long-term sequelae, which can include blindness, skin scarring and death. Additional considerations include the possibility for cross reactions if a person later uses a drug similar to the one in question, and whether family members share the same genetic susceptibility to such reactions, he adds.

Drug reaction lawsuits fall not under tort law, but under civil law, which puts them on a par with assault charges, Dr. Shear says. In lawsuits spawned by drug reactions, plaintiffs' lawyers must show not only that a physician gave the offending medication, but also that doing so violated a known standard of care. While this concept may seem vague, Dr. Shear says that legally, it means whatever is locally considered reasonable.

"Lawyers use phrases like 'the average prudent provider in a given community.' It's meant to be very contextual, based on one's specialty, experience and location. That doesn't define 'standard of care' completely. But it gives physicians some comfort in knowing that they're not being held to some hypothetical gold standard that doesn't exist," he says.

Reducing risks

Reducing risks up front requires keeping abreast of current findings regarding drug reactions, Dr. Shear says. For example, he says physicians who prescribe Tegretol (carbamazepine, Novartis) must know that "for the past few years, its label has stated that physicians should test for the HLA-B*1502 genotype in patients of Asian descent." This genotype occurs far more commonly than the background rate in the Han Chinese and other populations, such as those of Malaysian or Thai descent, he says.

Dr. Shear also recommends testing for this gene in patients whose family members have had hypersensitivity to carbamazepine or similar drugs. Other drugs that have been associated with SJS/TEN include nevirapine, lamotrigine, sertraline, pantoprazole, tramadol, sulfonamides, allopurinol and more (Mockenhaupt M, Viboud C, Dunant A, et al. J Invest Dermatol. 2008;128(1):35-44. Epub 2007 Sep 6).

A recent publication shows that people with skin detachment are most likely to experience septicemia (de Prost N, Ingen-Housz-Oro S, Duong T, et al. Medicine(Baltimore). 2010;89(1):28-36). "In two to three weeks, 50 percent of the people with more than 30 percent detachment begin to have bacteria in their blood," Dr. Shear says.

Should a drug reaction occur, priorities include properly diagnosing and treating it. The latter can be challenging because a recent retrospective study found insufficient evidence to recommend any particular approach (Schneck J, Fagot JP, Sekula P, et al. J Am Acad Dermatol. 2008;58(1):33-40. Epub 2007 Oct 4). "This review did suggest that corticosteroids show promise and require further study," Dr. Shear says. However, he adds that although pulling together disparate publications can be difficult, "I don't know if I agree with the corticosteroid recommendation."