Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma

Abstract

The aggressive epidermotropic CD8+ T-cell lymphoma or Berti's lymphoma, is a rare subtype of primary cutaneous lymphomas, first described in 1999. It is still considered a provisional entity by the latest World Health Organization classification of cutaneous lymphomas. Patients are commonly adults with a male predominance and it is characterized by widespread rapid evolving papules, hyperkeratotic annular plaques and ulcerated tumors. Histopathologic findings are characteristic, with an infiltrate of medium-sized CD8+ atypical lymphocytes involving the epidermis with a pagetoid pattern. It has an aggressive behavior and the prognosis is poor with a 5-year survival less than 40%. Instead these disease represents a therapeutic challenge, the initial treatment consists on polychemotherapy and hematopoietic stem cell transplantation.