Cancer Res Treat.  2010 Dec;42(4):239-243.

A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm Initially Mimicking Cutaneous Lupus Erythematosus

• Chang HJ
• Lee MD
• Yi HG
• Lim JH
• Lee MH
• Shin JH
• Choi SJ
• Moon Y
• Nahm CH
• Kim CS

Abstract

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.

Keyword

Plasmacytoid dendritic cells; Neoplasm; Drug therapy; Cutaneous lupus erythematosus

MeSH Terms

Aged
Arm
Biopsy
Bone Marrow
Dendritic Cells
Drug Therapy, Combination
Etoposide
Exanthema
Herpesvirus 4, Human
Humans
Lupus Erythematosus, Cutaneous
Lymph Nodes
Lymphocytes
Methotrexate
Methylprednisolone
Prednisolone
Prognosis
Rare Diseases
Skin
Thigh
Etoposide
Methotrexate
Methylprednisolone
Prednisolone

Figure

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  Fig. 1 Multiple nodules and plaques on the (A) cheek and (B) trunk.

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  Fig. 2 Findings of the initial skin biopsy. It shows scattered karyorrhectic debris and eosinophilic swelling of the dermal collagen fibers with a lymphocytic infiltration (×200).

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  Fig. 3 Findings of the second skin biopsy. (A) Shows an extensive diffuse infiltration of lymphoid cells (H&E, ×40). (B) Shows medium to large size lymphoid cells with nuclear atypia (H&E, ×200). (C) Shows the positive staining for CD4 and (D) shows the positive stain for CD56 on immunohistochemistry (×100).

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