Abstract
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare but aggressive malignancy with high mortality involving the skin and hematopoietic system. Clinical suspicion is difficult, and management of skin lesions is challenging due to their indolent course prior to dissemination. We describe a patient with isolated skin involvement who progressed to CD4+/CD56+ and CD123+ acute leukemia.