This kind of adverse drug reaction is caused by the accumulation of toxic metabolites; it is not the result of an IgE-mediated reaction. The risk of first-degree relatives developing the same hypersensitivity reaction is higher than in the general population.[1]
As this syndrome can present secondary to multiple anticonvulsants, the general term "anticonvulsant hypersensitivity syndrome" (AHS) is favored over the original descriptive term "dilantin hypersensitivity syndrome."[2]: 118 As of 2015, two cases of AHS have been reported that manifested during long-term treatment with multiple anti-seizure medications. Death due to multiple organ failure can occur; symptoms also mimic lymphoma and AHS has been called "pseudolymphoma" as a result. Changing the medications involved to those in a different class can avoid further problems.[5]