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Rapidly progressing papulonodular eruption in a middle-aged female - acute presentation of blastic plasmacytoid dendritic cell neoplasm

Introduction: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare, aggressive hematologic malignancy with frequent cutaneous involvement. It most commonly occurs in the elderly, with a male to female ratio of approximately 2.5-3:1. Case report: A 47- year- old female patient presented with a 3-week history of a papular rash involving her face, neck and upper trunk. She also reported having episodes of subfebrile temperatures throughout the last two years but was otherwise healthy. On physical exam, there were multiple dusky red to violaceous papules, primarily distributed on the forehead, perioral and submammary regions. A skin biopsy was performed with initial suspicion of sarcoidosis. Laboratory exams showed mild leukopenia and anemia. Within the following 10 days, the rash rapidly progressed with evident growth of the existing lesions and appearance of additional papules and tumoral nodules, at which point an additional skin biopsy as well as bone marrow biopsy were performed. Concurrently, there was progression of leukopenia with blasts detected in the peripheral blood. Initial skin biopsy showed dermal infiltration by medium-sized immature cells with irregular nuclei and scattered mitoses. The cells were positive for CD4, CD56, and CD123, and negative for CD34, MPO, CD3, CD68 and CD163, based on which a diagnosis of BPDCN was made. Second skin biopsy showed dermis expanded by sheets of atypical cells with the same immunohistochemical profile. These were also found in the bone marrow (trephine biopsy, immunophenotyping), indicating dissemination of the disease. Tumor cells showed cytogenetic abnormality on chromosome 8. Induction therapy based on the “7+3” chemotherapy regimen resulted in complete clinical remission including cutaneous lesions. However, after the first consolidation therapy (high dose cytarabine), a relapse occurred with bone marrow involvement and several new skin lesions. The patient received salvage chemotherapy based on the FLAG-IDA regimen, resulting in complete clinical, hematological and cytogenetic remission. The allogeneic stem cell transplantation is planned. Conclusion: This case illustrates the acute course of BPDCN, a rare hematologic malignancy with common initial presentation in the skin, undefined treatment guidelines and poor prognosis.

Blastic plasmacytoid dendritic cell neoplasm ; leukemia ; lymphoma

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