Behçet's syndrome associated with bullous necrotizing vasculitis

We recently saw a case of Behçet's syndrome in an 11-year-old Korean boy who had severe bullous necrotizing vasculitis as a skin manifestation. The patient exhibited three major criteria of the Shimizu classification, namely, oral and genital ulcerations, uveitis, and bullous necrotizing vasculitis as the skin manifestation. Immunologic laboratory data showed an increased OKT4/OKT8 ratio and a lymphocyte stimulation index with phytohemagglutinin. A skin specimen taken from a developing lesion showed features of lymphocytic vasculitis extending into the panniculus.