Carbamazepine-induced Stevens-Johnson syndrome in Chinese with negative HLA-A*3101 and HLA-B*1502 genes: A case report.

Stevens-Johnson syndrome (SJS) is a severe, potentially life-threatening disease primarily induced by medications, which often involves lesions of the oral mucosa, eyes and skin. Its pathogenesis is partially associated with genetic factors. The present study reported a case of SJS following oral administration of carbamazepine (CBZ). A total of ~2 weeks after initiating the medication, the patient developed generalized skin rashes with pruritus, which progressively evolved into patchy erythema on the face, neck, chest, back, oral cavity and lips; oral herpes also emerged. As the condition advanced, the patient experienced blurred vision, along with rupture and exudation of the oral and labial herpes. The patient was diagnosed with SJS. However, genotyping results revealed a negative status for both major histocompatibility complex, class I, A (HLA-A)*3101 and HLA-B*1502 genes, indicating that the risk of SJS induced by CBZ cannot be excluded, even if the genetic test yields negative results. After confirmation of the diagnosis, CBZ was discontinued and the patient received treatment including intravenous dexamethasone. Significant clinical improvement was observed within ~10 days. The present study emphasized that the potential risk of CBZ-associated SJS cannot be ignored, despite negative results for both HLA-A*3101 and HLA-B*1502.