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Carbamazepine (CBZ) is one of commonly used drugs for the treatment of trigeminal neuralgia.However, it can cause many forms of hypersensitivity reactions, from mild skin reactions to severe cutaneous adverse reactions (Steven-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)), which led up to approximately 10%-30% of death.Chung from Taiwan were the first to find association between CBZ-induced SJS/TEN and HLA-B*1502.Recently studies have proved similar results in mainland southern China, but not in mainland northern China.The aim of this research is to test whether there is an association between HLA-B*4601and CBZ-induced cutanous adverse reactions among mainland northern Han-Chinese.HLA-B*4601 genotyping was performed by sequence-specific primer polymerase chain reaction.HLA-B*4601 allele was present in 37.5%(3/8) of SJS/TEN patients, 29.4% (5/17) of MPE patients, 5.5% (2/36) of tolerant patients, and 8% (4/50) of healthy controls.The frequency of HLA-B*4601 allele in CBZ-induced SJS/TEN patients was significantly higher (P<0.05)compared with the tolerant group and also the same as control group.Our results suggest that HLA-B*4601 might be associated with CBZ-induced SJS/TEN among the mainland northern Han-Chinese population.