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Angiocentric cutaneous T-cell lymphoma of childhood (hydroa-like lymphoma): A distinctive type of cutaneous T-cell lymphoma

1998; Elsevier BV; Volume: 38; Issue: 4 Linguagem: Inglês

10.1016/s0190-9622(98)70120-3

1097-6787

Mario Magaña, P Sangüeza, Javier Gil-Beristain, Sergio Sánchez-Sosa, Angeles Salgado, G Ramon, Omar P. Sangüeza,

Lymphoma Diagnosis and Treatment

Abstract Background: Angiocentric cutaneous T-cell lymphomas of childhood (ACTCLC) are an unusual type of T-cell lymphomas that present with a vesiculopapular eruption mimicking hydroa vacciniforme. Most patients have been children from Asia and Latin America. Objective: The purpose of this study was to describe four cases of ACTCLC; to discuss its clinical, histopathologic, and immunohistochemical features; to consider its possible relationship to the Epstein-Barr virus (EBV); and to clarify its classification within the spectrum of angiocentric lymphomas. Methods: The clinical, histopathologic, and immunohistochemical features of four cases of ACTCLC were identified and analyzed. In addition in situ hybridization for EBV was performed in all cases. Results: The clinical features were similar to previous cases reported under different names, such as hydroa-like lymphomas, edematous, scarring vasculitic panniculitis. Histologically, all showed angiocentric infiltrates composed mainly of T cells. In all cases there were variable numbers of CD30 + cells. The EBV was present in three of the cases. Conclusion: ACTCLC 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. (J Am Acad Dermatol 1998;38:574-9.)