Lamotrigine-induced Stevens-Johnson syndrome in the setting of mitochondrial encephalomyopathy: A case report

A 67-year-old male with Mitochondrial Encephalomyopathy with Lactic Acidosis and Stroke-Like Episodes (MELAS) who developed life-threatening Stevens-Johnson Syndrome (SJS) more than one month after initiating lamotrigine (LTG) for epilepsy management. Despite prompt LTG discontinuation and aggressive immunomodulatory therapy, the patient succumbed to severe infection and multiple organ failure. LTG-induced SJS is associated with multiple risk factors. The immune-activating microenvironment resulting from mitochondrial dysfunction may potentiate the risk of LTG-induced SJS. Lamotrigine should be used cautiously for epilepsy in high-risk patients, particularly those with mitochondrial encephalomyopathy (ME), and comprehensive evaluation is required to minimize severe hypersensitivity reactions.