The Journal of Bucharest College of Physicians and the Romanian Academy of Medical Sciences

An Unusual Presentation of Primary Cutaneous Aggressive Epidermotropic CD8+ T Cell Lymphoma


Primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma is an extremely rare type of cytotoxic lymphoma reported for the first time by Berti et al. in 1999, (1) who described the tumor as a distinct clinico-pathologic entity with an aggressive clinical course. It presents as widespread, rapidly evolving papules, plaques, and tumors, often showing central necrosis and ulceration, histologically characterized by epidermotropism of CD8+ CD4- T cells. The condition has an increased tendency of spreading to extranodal sites, usually responds poorly to conventional therapies for classic CD4+ cutaneous T cell lymphomas (CTCLs), and has an unfavorable prognosis. (1-4)