Putative pathogenesis of HV-LPD. Individuals with a particular genetic background after primary EBV infection develop a chronic state characterized by the presence of EBV-infected T cells. Circulating EBV-infected T or NK cells are recruited to the skin and activated following sun exposure. Local production of IFN-γ and chemokines results in inflammation and tissue damage. The skin biopsy shows epidermal reticular degeneration leading to intraepidermal spongiotic vesiculation. The lymphoid infiltrate predominates in the dermis around adnexae and blood vessels. The lymphoid cells are positive for EBV (in black), as demonstrated by in situ hybridization for EBV-encoded small RNA. Due most probably to additional genetic, immunological, and/or environmental factors, a subset of patients mostly of Asian and Hispanic ethnicity may develop severe disease with systemic involvement, whereas the remainder show an indolent, self-limiting course. Rare patients with an indolent presentation may progress after years to the more severe form of HV-LPD with systemic symptoms. HV-LPD with systemic symptoms has been referred in the literature as “severe” HV or HV-like lymphoma. Tx, treatment.