Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy derived from plasmacytoid dendritic cells. The incidence is unknown due to its rarity; however, among the cutaneous lymphomas, it represents less than 1% of cases. Most reported cases are in adults, with a median age of 65-67 years at diagnosis.

BPDCN usually has an initial cutaneous presentation with or without bone marrow involvement and rarely presents as a leukemia. The skin manifestations are red, brown, or violaceous papules, plaques, nodules, or tumors that may be localized or widespread. The presence of eruptive cutaneous lesions portends a worse prognosis. Extracutaneous involvement has been reported in tonsils, paranasal cavities, lungs, eyes, and the central nervous system. Patients may have cytopenias, lymphadenopathy, and/or splenomegaly as well.