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Published on: November 4, 2016
Blastic plasmacytoid dendritic cell neoplasm presenting as fever with diffuse cutaneous nodules
C V Dincy Peter1, Anupam Chakrapani, Sanjeev Shah
1Department of Dermatology, Venereology and Leprosy, Christian Medical College, Vellore, India. dincypeter@gmail.com
Indian Journal of Dermatology
|April 4, 2012
Summary
Blastic plasmacytoid dendritic cell neoplasm is a rare condition presenting with skin lesions and potential systemic involvement. Early diagnosis through skin biopsy and immunohistochemistry is crucial for effective management.
Area of Science:
- Oncology
- Dermatology
- Hematology
Background:
- Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy.
- It commonly presents with cutaneous involvement, often with extracutaneous disease.
Purpose of the Study:
- To present a case of BPDCN in a young man with disseminated skin lesions.
- To highlight the diagnostic features and immunohistochemical profile of BPDCN.
Main Methods:
- Clinical presentation of a young male with fever and skin lesions.
- Dermatopathological examination including light microscopy and immunohistochemistry (CD4, CD8, CD56, CD30, TdT, Alk-1).
- Evaluation of lymph node and bone marrow for neoplastic infiltration.
Main Results:
- The patient presented with high-grade fever and disseminated skin papules and nodules.
- Microscopic examination revealed atypical lymphoid cells positive for CD4, CD8, and CD56.
- Bone marrow involvement confirmed systemic disease, leading to a diagnosis of BPDCN.
Conclusions:
- BPDCN is a rare neoplasm that can manifest primarily in the skin.
- Immunohistochemistry is essential for diagnosing BPDCN, distinguishing it from other lymphoid neoplasms.
- The case underscores the importance of considering BPDCN in patients with unexplained skin lesions and systemic symptoms.
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