[Blastic plasmacytoid dendritic cell neoplasm revealed by ecchymotic lesions on the face].
BACKGROUND
Cutaneous CD4+CD56+ malignant tumor proliferation was previously called "CD4/CD56 hematodermic neoplasm". However, the most recent studies have shown that the disease develops from plasmacytoid dendritic cells and the tumor has been renamed "Blastic Plasmacytoid Dendritic Cell Neoplasm" (BPDCN). It is an aggressive disease with a poor prognosis and behaves like acute leukemia in the short to moderate term.


METHODS
A 65-year-old man with no particular history consulted for a left laterocervical lesion of ecchymotic aspect that had appeared one year earlier. Topical corticosteroid therapy had been unsuccessful. Examination of biopsies with lymphocyte typing enabled a diagnosis of BPDCN to be made. At the histopathological level, biopsy showed an infiltrate comprising medium to large cells. Immunohistochemical examination was remarkable for the absence of expression of markers of T- and B-cell lines. However, these tumor cells expressed CD4, CD56 and TCL1. Staging of the disease was normal. Treatment with chemotherapy was initiated in collaboration with a team of hematologists. Autologous bone marrow transplant was then performed.


CONCLUSIONS
BPDCN is a rare malignant blood dyscrasia. It is distinguished by inaugural skin involvement, with systemic manifestations occurring much later. Histopathological examination of a skin biopsy with immunostaining establishes the diagnosis. In terms of phenotype, the tumor population is highly characteristic. The cells are negative for antigens of T- and B- cell lines. However, these cells express CD4, CD56 and TCL1, which are markers of plasmacytoid dendritic cells. The disease carries a poor prognosis and evolves in the short to middle term in the same way as acute leukemia. First-line treatment consists of the chemotherapy regimens used in aggressive lymphoma or acute leukemia. A bone marrow graft is sometimes performed at the time of initial relapse. Average survival is 12 months for chemotherapy alone and 30 months for transplant after first relapse. Early bone marrow transplantation has been shown to improve survival.
Service de dermatologie, hôpital Saint-Vincent, boulevard de Belfort, BP 387, 59020 Lille cedex, France; Service de dermatologie, CHU de Treichville-Abidjan, BP V3 Abidjan, Côte d'Ivoire. Electronic address: ahogoceleste@yahoo.fr.
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Ahogo
Service de dermatologie, hôpital Saint-Vincent, boulevard de Belfort, BP 387, 59020 Lille cedex, France.
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Wantz
Service d'hématologie, hôpital Saint-Vincent, boulevard de Belfort, BP 387, 59020 Lille cedex, France.
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Cliquennois
Service d'anatomie pathologique, hôpital Saint-Vincent, boulevard de Belfort, BP 387, 59020 Lille cedex, France.
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Gosset
Service de dermatologie, hôpital Saint-Vincent, boulevard de Belfort, BP 387, 59020 Lille cedex, France.
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Lebas
Service de dermatologie, hôpital Saint-Vincent, boulevard de Belfort, BP 387, 59020 Lille cedex, France.
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Modiano
D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001215:Asparaginase / Q000008:administration & dosage; D014408:Biomarkers, Tumor; D001706:Biopsy; D016026:Bone Marrow Transplantation; D015704:CD4 Antigens / Q000032:analysis; D019002:CD56 Antigen / Q000032:analysis; D003131:Combined Modality Therapy; D003713:Dendritic Cells / Q000473:pathology*; D003907:Dexamethasone / Q000008:administration & dosage; D004438:Ecchymosis / Q000209:etiology*; D005148:Facial Dermatoses / Q000209:etiology*; D019337:Hematologic Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D011518:Proto-Oncogene Proteins / Q000032:analysis; D014182:Transplantation, Autologous
D014408:Biomarkers, Tumor; D015704:CD4 Antigens; D019002:CD56 Antigen; C522918:NCAM1 protein, human; D011518:Proto-Oncogene Proteins; C090851:TCL1A protein, human; D003907:Dexamethasone; D001215:Asparaginase; D008727:Methotrexate
Bruising; CD4; CD56; Cellules plasmacytoïdes; Chemotherapy; Chimiothérapie; Ecchymose; Hematodermic tumor; Hématodermie; Immunophenotyping; Immunophénotypage; Plasmacytoid cells