Abstract
The Stevens-Johnson syndrome (SJS) and the drug rash with eosinophilia and systemic symptoms (DRESS) are part of a spectrum of severe adverse cutaneous drug reactions. It is prudent to differentiate between the two syndromes, as the management and prognosis are different. Carbamazepine is one of the common culprit drugs for both SJS and DRESS. This case study is of a 45-year-old Indonesian women who developed atypical presentation of SJS following carbamazepine therapy. However, it is essential to distinguish between the SJS and DRESS. Moreover, carbamazepine-induced SJS is strongly linked to specific human leukocyte antigen alleles in the South Asian people. Henceforth, this case emphasizes the importance of differentiating SJS from DRESS and substantiates other studies that strongly recommend testing for human leukocyte antigen alleles before initiating carbamazepine in a susceptible population.