Hydroa Vacciniforme Is Associated with Increased Numbers of Epstein–Barr Virus–Infected γδT Cells

Abstract

Hydroa vacciniforme (HV) is a rare photosensitivity disorder of childhood associated with Epstein-Barr virus (EBV)(+) T-cell infiltration. We have summarized clinical manifestations of HV, and analyzed EBV(+) T-cell subsets as well as EBV DNA load in lymphocyte fractions, in comparison with hypersensitivity to mosquito bites (HMB), an EBV-associated T/natural killer (NK) lymphoproliferative disorder. We found that 31 of 33 (93.9%) HV lesions were composed of EBV(+) T cells and reactive EBV(-) cytotoxic T cells, without significant CD56(+) cell infiltration, whereas many CD56(+) cells were present in 8 of 9 (88.9%) HMB lesions. Of 13 (20.6%) HMB patients with or without HV, 12 (92.3%) showed increased percentages (>32%) of NK cells in the peripheral blood, whereas in the 16 patients with HV alone, 14 (87.5%) showed no increase. Of the 11 HV patients, 10 (90.9%) had increased percentages (>5%) of circulating γδT cells, with a mean value of 15.7 ± 2.9%, and the γδT-cell fractions contained larger amounts of EBV DNA than non-γδT-cell fractions. A triple-labeling method revealed that all three HV patients examined had increased percentages of EBER(+), T-cell receptor (TCR)γδ(+), and TCRαβ(-) cells. Our observations indicate that HV is associated with increased numbers of EBV(+) γδT cells, whereas HMB is associated with EBV(+) NK cells.

Application of flow cytometric in situ hybridization assay to Epstein–Barr virus‐associated T/natural killer cell lymphoproliferative diseases

“…This observation accords with other recent reports. (43,44) The cd T cells are the major T cell population in the skin and mucosal epithelium. The cd T cells secrete various cytokines and have cytolytic properties.…”

Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma

“…15 Recently it has been reported that HV and/or HVLL are associated with increased numbers of EBV-infected gd T cells in peripheral blood. 24,30,31 Kimura et al 24 demonstrated that the EBV-infected cells in peripheral blood in almost half of HVLL patients (6 of 13 cases) were gd T cells. Nevertheless, in none of Case CD3 CD4 CD8 TIA-1 CD56 CD30 BF1 EBER LMP EBNA2 TCR-d TCR-g Lineage BLOOD, 31 OCTOBER 2013 x VOLUME 122, NUMBER 18 HYDROA VACCINIFORME-LIKE LYMPHOMA 3107…”

Sunlight‐induced papulovesicular eruption in an 8‐year‐old girl

“…Die histopathologische Untersuchung zeigt eine epidermale Nekrose mit intraepidermaler Blasenbildung. Für diese Verläufe ist eine Assoziation mit einer latenten oder chronisch aktiven EBV-Infektion gut dokumentiert [9,10]. Zudem ist eine Beteiligung von Mundschleimhaut, Gastrointestinaltrakt und Auge beobachtet worden [5,8].…”