Recurrent erythema multiforme major in an 8-year-old patient with recurrence of herpetic gingivostomatitis and HLA-B*5801 haplotype: A causal or casual relationship?

Erythema multiforme (EM) is an immune-mediated reaction first described by von Hebra1 in 1860, presenting typically as acrofacial target lesions. The rash may be confined to the skin (EM minor), or it may involve ocular, oral, or genital mucosa with systemic symptoms (EM major). In most cases, only 1 outbreak of EM occurs in a lifetime; however, very rarely, repeated episodes of EM may occur, known as recurrent EM.2 Few cases of pediatric recurrent EM have been reported, with the etiology being identified in less than half of these cases.3 We report a pediatric case of recurrent EM resistant to conventional treatments in which a peculiar human leukocyte antigen (HLA) haplotype has been identified.