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.
References
- Guang Liu. 2023. Blastic plasmacytoid dendritic cell neoplasm (BPDCN). Cases in Laboratory Genetics and Genomics (LGG) Practice, 269-273. https://doi.org/10.1016/B978-0-323-99622-8.00032-0
- Naveen Pemmaraju, Nathaniel R. Wilson, Guillermo Garcia-Manero, Koji Sasaki, Joseph D. Khoury, Nitin Jain, Gautam Borthakur, Farhad Ravandi, Naval Daver, Tapan Kadia, Courtney DiNardo, Elias Jabbour, Sherry Pierce, Muzaffar Qazilbash, Marina Konopleva, Hagop Kantarjian. (2022) Characteristics and outcomes of patients with blastic plasmacytoid dendritic cell neoplasm treated with frontline HCVAD. Blood Advances 6:10, 3027-3035. https://doi.org/10.1182/bloodadvances.2021006645