Blastic Plasmacytoid Dendritic-Cell Neoplasm

This article is an answer to the Case – Painless, Nonpruritic, Volaceous Skin Lesions

Immunohistochemical analysis showed cells that were positive for CD4, CD56, and CD123, which supported the diagnosis of blastic plasmacytoid dendritic-cell neoplasm, a rare hematologic cancer.

Computed tomographic scans did not reveal evidence of extracutaneous disease. Bone marrow aspiration showed the presence of 1% neoplastic plasmacytoid dendritic cells.

The patient received a 1-week course of dexamethasone and had a substantial reduction in the cutaneous lesions. He was then treated with eight cycles of hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone) alternating with high-dose methotrexate and cytarabine, a course of therapy that resulted in complete remission.

Blastic plasmacytoid dendritic-cell neoplasm is a systemic disease, although it can manifest with cutaneous lesions. It is important to obtain complete staging evaluation before the initiation of therapy, including the use of glucocorticoids.