A Case of Primary Cutaneous Aggressive Epidermotropic CD8+ Cytotoxic T-Cell Lymphoma

Abstract

Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma is an aggressively proliferating cutaneous lymphoma expressing CD8+ cytotoxic phenotype. A 61-year-old man presented with a 1-year history of extensively diffuse erythematous plaques on the trunk, upper extremities, and face which had central necrosis with oozing and crust formation. Histopathologic examination displayed diffuse aggregates of atypical lymphoid cells with a pagetoid spreading of epidermotropism. The proliferating lymphocytes indicated strong labelling with CD3, CD8, and were negative for CD4, CD20 and CD56. Molecular analysis exhibited monoclonal rearrangement of TCR genes. Based on these clinical and histologic profiles, the patient was finally diagnosed with primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma. This disease, classified as a provisional entity belonging to cutaneous T-cell lymphoma, has been reported in less than 60 case reports. Herein, we described the first Korean case of primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, characterized by distinct clinicopathological and hard-to-cure features.