BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematodermic malignancy neoplasm with highly aggressive course and poor prognosis. This disease typically presents with cutaneous involvement as the first manifestation, with subsequent or simultaneous spread to bone marrow and peripheral blood. 

CASE REPORT: Here, we report the case of a 51-year-old woman who presented a violaceus skin lesion on the lateral region of the right thigh, weight loss, fever, and lymphadenopathies. Computed tomography (CT) displayed thoracic and abdominal lymph node and alveolar bleeding. Flow cytometry from circulating blastic cells was compatible with BPDCN (CD4+, CD56+ and CD123+). She underwent 5 cycles of hyper-CVAD alternating with high-dose methotrexate and cytarabine, but the patient died due to alveolar bleeding and sepsis.

CONCLUSIONS: We report a rare case of BPDCN characterized by an aggressive course, presence of atypical skin lesion, a finding suggestive of pulmonary infiltration, and nonresponse to induction chemotherapy, leading to late diagnosis and therapeutic management. Because of the late recognition of the skin lesion, neoplastic cells infiltrated the dermis and spread as the disease progressed rapidly to a fatal course.

Keywords: Leukemia, Myeloid, Acute, Lung Compliance, Rare Diseases, Skin Neoplasms