抄録
Hydroa vacciniforme (HV) is a chronic photosensitivity disorder. Here, we report the case of a three-year-old Japanese girl with HV-like lymphoproliferative disorders. After sun exposure, she presented with low-grade fever and typical HV-like eruption. High numbers of EBV genomes were found within peripheral blood cells. In situ hybridization (ISH) on skin biopsy samples demonstrated that 25% of the lymphocytes infiltrating the skin contained EBV-encoded small nuclear RNA (EBER-1), while ISH of peripheral blood cells demonstrated that 21% of lymphocytes expressed EBER-1. Most γδ T cells were EBER-1+ and activated, whereas almost no CD4+ T, CD8+ T, or NK cells were positive for EBER-1. Although we could not identify the skin infiltrating EBER-1+ T cells as γδ T cells by immunohistochemistry, the CD3+CD4-CD8- cells identified in the skin were assumed to be of a similar subset as the peripheral blood EBER-1+ clonal γδ T cells. We diagnosed the patient as having HV-like eruptions associated with γδ T cell-CAEBV. She was treated by chemotherapy and allogeneic cord blood transplantation. Since transplantation the patient has not relapsed the skin eruption and EBV genome has not been detected to date. Our case indicates that chronic EBV infection of γδ T cells can induce typical HV eruption.
