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

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

Affiliations
  • 1Department of Internal Medicine, Inha University Hospital, Incheon, Korea. cskimmd@inha.ac.kr
  • 2Department of Dermatology, Inha University Hospital, Incheon, Korea.
  • 3Department of Pathology, Inha University Hospital, Incheon, Korea.
  • 4Department of Laboratory Medicine, Inha University Hospital, Incheon, Korea.

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

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

  • 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).

  • 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).


Reference

1. Adachi M, Maeda K, Takekawa M, Hinoda Y, Imai K, Sugiyama S, et al. High expression of CD56 (N-CAM) in a patient with cutaneous CD4-positive lymphoma. Am J Hematol. 1994; 47:278–282. PMID: 7526680.
Article
2. Bueno C, Almeida J, Lucio P, Marco J, Garcia R, de Pablos JM, et al. Incidence and characteristics of CD4(+)/HLA DRhi dendritic cell malignancies. Haematologica. 2004; 89:58–69. PMID: 14754607.
3. Oshimi K, Kawa K, Nakamura S, Suzuki R, Suzumiya J, Yamaguchi M, et al. NK-cell neoplasms in Japan. Hematology. 2005; 10:237–245. PMID: 16019472.
Article
4. Kim Y, Kang MS, Kim CW, Sung R, Ko YH. CD4+CD56+ lineage negative hematopoietic neoplasm: so called blastic NK cell lymphoma. J Korean Med Sci. 2005; 20:319–324. PMID: 15832009.
5. Lee M, Ko Y, Kim K, Kim M, Kim M, Cho K. Clinicopathologic and genetic study of cutaneous blastic NK lymphoma in Korea. Korean J Dermatol. 2007; 45(suppl 2):145.
6. Petrella T, Dalac S, Maynadié M, Mugneret F, Thomine E, Courville P, et al. Groupe Français d'Etude des Lymphomes Cutanés (GFELC). CD4+CD56+ cutaneous neoplasms: a distinct hematological entity? Am J Surg Pathol. 1999; 23:137–146. PMID: 9989839.
7. Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005; 105:3768–3785. PMID: 15692063.
Article
8. Chaperot L, Bendriss N, Manches O, Gressin R, Maynadie M, Trimoreau F, et al. Identification of a leukemic counterpart of the plasmacytoid dendritic cells. Blood. 2001; 97:3210–3217. PMID: 11342451.
Article
9. Jaye DL, Geigerman CM, Herling M, Eastburn K, Waller EK, Jones D. Expression of the plasmacytoid dendritic cell marker BDCA-2 supports a spectrum of maturation among CD4+ CD56+ hematodermic neoplasms. Mod Pathol. 2006; 19:1555–1562. PMID: 16998465.
Article
10. Cota C, Vale E, Viana I, Requena L, Ferrara G, Anemona L, et al. Cutaneous manifestations of blastic plasmacytoid dendritic cell neoplasm-morphologic and phenotypic variability in a series of 33 patients. Am J Surg Pathol. 2010; 34:75–87. PMID: 19956058.
Article
11. Rönnblom L, Pascual V. The innate immune system in SLE: type I interferons and dendritic cells. Lupus. 2008; 17:394–399. PMID: 18490415.
Article
12. Jegalian AG, Facchetti F, Jaffe ES. Plasmacytoid dendritic cells: physiologic roles and pathologic states. Adv Anat Pathol. 2009; 16:392–404. PMID: 19851130.
13. Vermi W, Lonardi S, Morassi M, Rossini C, Tardanico R, Venturini M, et al. Cutaneous distribution of plasmacytoid dendritic cells in lupus erythematosus. Selective tropism at the site of epithelial apoptotic damage. Immunobiology. 2009; 214:877–886. PMID: 19625100.
Article
14. Leitenberger JJ, Berthelot CN, Polder KD, Pro B, McLaughlin P, Jones D, et al. CD4+ CD56+ hematodermic/plasmacytoid dendritic cell tumor with response to pralatrexate. J Am Acad Dermatol. 2008; 58:480–484. PMID: 18280345.
Article
15. Dalle S, Beylot-Barry M, Bagot M, Lipsker D, Machet L, Joly P, et al. Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice? Br J Dermatol. 2010; 162:74–79. PMID: 19689477.
Article
Full Text Links
  • CRT
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr