Abstract: Blastic plasmacytoid dendritic cell tumor is extremely malignant, the skin is the first manifestation of the disease, and the manifestations of skin lesions are diverse, which is easy to be misdiagnosed and missed diagnosed. The diagnosis of this disease requires a multiple immunophenotypys and histopathological features. It is prone to relapse and has a poor prognosis. A 76-year-old male patient presented with dark-purple invasive nodules and masses on the head and neck as the first manifestation. The immunophenotypic and histopathological features were in accordance with blastic plasmacytoid dendritic cell tumor.

Key words: blastic plasmacytoid dendritic cell neoplasm, skin neoplasms, immunophenotype, clinicopathologic features