Clinical and immunopathological analysis was performed on 24 cases of Sjögren syndrome with annular erythema (AESjS). AESjS predominantly appears on the cheek of the face where skin temperature is relatively low in comparison with other sites. VCAM-1 and ICAM-1 were strongly expressed on endothelial cells of AESjS, while epidermal expression of ICAM-1 was focal and weak. VCAM-1 mRNA expression was also much more intense compared to systemic lupus erythematosus. The lymphocyte response to staphylococcal enterotoxin B was higher in AESjS than that of controls, and cells positive for T cell receptor Vβ6,9,12 were expanded after the culture. Superantigen-driven endothelial-cell-dependent T cell infiltration to the skin plays a crucial role in AESj