Bruise-like cutaneous lesions as the early presentation of blastic plasmacytoid dendritic cell neoplasm

• Main Authors: Yi-Hsuan Chou, Ruey-Yi Lin, Meng-Sui Lee, Hsiao-Ping Wu, Che-Pin Lin, Chen-Yi Wu
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2014-06-01
• Series: Dermatologica Sinica
• Subjects: blastic natural-killer cell lymphoma; blastic plasmacytoid dendritic cell neoplasm; CD4+CD56+ hematodermic neoplasm
• Online Access: http://www.sciencedirect.com/science/article/pii/S1027811713000633

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare and highly aggressive hematopoietic malignancy associated with a poor prognosis. It has been recognized to originate from precursors of plasmacytoid dendritic cells and has recently been established as a distinct entity. The most frequent clinical presentations are prominent skin lesions, followed by peripheral blood, bone marrow, and other organ involvement. Treatment outcomes are often disappointing due to a high relapse rate after chemotherapy and risk of leukemic dissemination. Herein, we report a case of BPDCN in a 41-year-old man who presented with cutaneous and nasopharyngeal lesions, and achieved a complete response after systemic chemotherapy. Dermatologists should be aware of BPDCN and its clinical presentations, as early diagnosis with appropriate treatment is crucial to improve its prognosis.