Epstein-Barr virus-associated hydroa vacciniforme-like cutaneous lymphoma in seven Chinese children

Hydroa vacciniforme-like cutaneous lymphoma is a rare type of Epstein-Barr virus-associated lymphoma. We analyze clinicopathologic features of seven Chinese child patients with hydroa vacciniforme-like cutaneous lymphoma and determine the pathogenic association of Epstein-Barr virus with this disorder. Clinical, histologic, and immunohistochemical features were reviewed. Skin lesions were subjected to Epstein-Barr virus-encoded RNA detection by in situ hybridization. Serologic assay and quantitation of Epstein-Barr virus DNA were performed. All seven patients presented with facial vesicles, papulovesicles, and atrophic scarring. Histologically, skin specimens showed epidermal blister formation, and dense lymphoid infiltration throughout the dermis and subcutaneous tissue. The infiltrate was composed of both small and large irregular lymphocytic cells expressing CD45RO or CD56. Tumor cells positive for Epstein-Barr virus-encoded RNA were detected in cutaneous infiltrates in all seven cases. Besides skin eruptions, all patients had systemic manifestations, such as intermittent fever, hepatosplenomegaly or lymph node enlargement. The amounts of Epstein-Barr virus DNA were increased in the peripheral blood in two detected cases, and antibody titers to Epstein-Barr virus revealed a chronic active Epstein-Barr virus infection in all cases. The diagnosis of hydroa vacciniforme-like cutaneous lymphoma was made. Four of seven patients were treated with interferon α intramuscularly, and the skin eruptions improved. Hydroa vacciniforme-like cutaneous lymphoma may present with severe facial edema, papulovesicular eruptions and permanent scars. The tumor cells often express natural killer- or T-cell markers. The disease is often preceded by chronic active Epstein-Barr virus infection.