Blastic Plasmacytoid Dendritic Cell Neoplasm --a Rapidly Evolving Entity. Case Report.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN), CD4+/CD56+hematodermic neoplasm was formally known as blastic NK-cell lymphoma. It is in fact a form of acute myeloid leukemia notable for highly aggressive behavior with cutaneous, lymph node and bone marrow involvement. This entity is derived from plasmocytoid dendritic cells and has a predilection for extranodal sites, especially the skin. Elderly male patients are the most affected and the prognostic is poor. The first case was reported in 1994 and sice then, single cases and a few small series have been published. This article presents the case of a previously healthy 56-years-old man, who presented himself to a skin eruption consisting in multiple, large dermal ulcerated tumors, located on the trunk and scalp. The lesions were painless and grew in size rapidly. Physical examination was normal except for the skin lesions. Histological examination of a biopsy specimen and immunohistochemical studies (positive for next markers: CD4, CD 45, CD56, CD68, Ki 67) revealed the rare diagnostic-blastic plasmacytoid dendritic cell neoplasm.