Abstract: A case of hydroa vacciniforme-like lymphoma was reported and the literature of the latest WHO classification, EBV infection-related pathogenesis, differential diagnosis and treatment were reviewed. A 38-year-old male presented with relapsed generalized lesions and fever for 30 years and aggravated for 1 year. Pitted scars were left after lesions relieved. The patient was positive for EB antibodies (IgG). Histopathological examination showed dense infiltration of lymphocytes in the whole dermis and subcutaneous tissue. Immunohistochemistory showed CD2(+++), CD3(++), CD8(++), TIA-1(++), CD56(+), CD20(focal +) , Ki-67＞30%, EBER(+). The diagnosis of hydroa vacciniforme-like lymphoma was made.

Key words: hydroa vacciniforme-like lymphoma, hydroa vacciniforme-like lymphoproliferative disorder, Epstein-Barr virus