ANGIOCENTRIC CUTANEOUS T-CELL LYMPHOMA OF CHILDHOOD (HYDROA-LIKE LYMPHOMA) - A DISTINCTIVE TYPE OF CUTANEOUS T-CELL LYMPHOMA

Background: Angiocentric cutaneous T-cell lymphomas of childhood (ACTC LC) are an unusual type of T-cell lymphomas that present with a vesicu lopapular eruption mimicking hydroa vacciniforme. Most patients have b een children from Asia and Latin America. Objective: The purpose of th is study was to describe four cases of ACTCLC; to discuss its clinical , histopathologic, and immunohistochemical features; to consider its p ossible relationship to the Epstein-Barr virus (EBV); and to clarify i ts classification within the spectrum of angiocentric lymphomas. Metho ds: The clinical, histopathologic, and immunohistochemical features of four cases of ACTCLC were identified and analyzed. In addition in sit u hybridization for EBV was performed in all cases. Results: The clini cal features were similar to previous cases reported under different n ames, such as hydroa-like lymphomas, edematous, scarring vasculitic pa nniculitis. Histologically, all showed angiocentric infiltrates compos ed mainly of T cells. In all cases there were variable numbers of CD30 (+) cells. The EBV was present in three of the cases. Conclusion: ACTC LC is a distinct type of T-cell lymphoma. It affects mainly children, and the EBV appears to play a role in the pathogenesis of this disease .