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Blastic plasmacytoid dendritic cell neoplasm: report of a case presenting with lung and central nervous system involvement and review of the literature.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic malignancy with almost invariably cutaneous involvement and poor prognosis. We report a case of BPDCN in a 58-year-old man who presented with skin, lymph node, bone marrow, peripheral blood, lung, and central nervous system involvement. To the best of our knowledge, central nervous system (CNS) involvement as initial presentation has not been reported since the latest World Health Organization (WHO) classification of tumors of the hematopoietic and lymphoid tissues in 2008. Review of the literature was performed on BPDCN cases published in 2008-2013 in PubMed. The major clinical, histopathologic, immunophenotypic, and cytogenetic aspects of the disease were discussed. Dermatologists and dermatopathologists should be aware of this rare disease for which nearly half of the patients present with only cutaneous lesions at diagnosis, as it may allow for early diagnosis and appropriate treatment.