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KMID : 0939920100420040239
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대한암학회지
2010 Volume.42 No. 4 p.239 ~ p.243
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A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm Initially Mimicking Cutaneous Lupus Erythematosus
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Chang Hye-Jung
Lee Myung-Dong
Yi Hyeon-Gyu
Lim Joo-Han
Lee Moon-Hee
Shin Jeong-Hyun
Choi Suk-Jin
Moon Yeon-Sook
Nahm Chung-Hyun
Kim Chul-Soo
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Abstract
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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare disease. The prognosis is poor in most cases with rapid progression despite administering chemotherapy. A 67-year-old man complained of skin rashes on his back and this spread to the trunk, face, arms and thighs, and he was initially diagnosed with cutaneous lupus erythematosus according to the skin biopsy. The skin rashes then became aggravated on a trial of low dose methylprednisolone for 3 months. Repeated skin biopsy revealed a diffuse infiltration of lymphoid cells with medium sized nuclei, positive for CD4 and CD56, negative for Epstein-Barr virus (EBV), indicating a diagnosis of BPDCN. Further workups confirmed stage IVA BPDCN involving the skin, multiple lymph nodes, the peripheral blood and the bone marrow. He was treated with six cycles of combination chemotherapy consisting of ifosphamide, methotrexate, etoposide, prednisolone and L-asparaginase, and he achieved a partial response. Herein we report on a rare case of BPDCN that was initially misinterpreted as cutaneous lupus erythematosus.
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KEYWORD
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Plasmacytoid dendritic cells, Neoplasm, Drug therapy, Cutaneous lupus erythematosus
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