title: Leukaemic cutaneous T-cell lymphoma-manifesting papuloerythroderma with CD3(-) CD4(+) phenotype.
abstract: The leukaemic form of cutaneous T-cell lymphoma, as represented by Sézary syndrome, exhibits erythroderma. We describe here an indolent leukaemic patient with cutaneous T-cell lymphoma, who initially had a nodulo-tumourous eruption with a crop of solid papules, but finally presented with papuloerythroderma. Histologically, the skin lesions showed non-epidermotropic dermal infiltration of atypical lymphocytes with lymphoid follicles and a granulomatous change. The circulating malignant CD4(+) CCR4(+) T cells lacked the expression of T-cell receptor and did not respond to concanavalin A. The unresponsiveness of T cells to the T-cell mitogen may be associated with the non-epidermotropic behaviour of the tumour cells and the initially non-erythrodermic eruption.
authors.affiliation: Department of Dermatology, University of Occupational and Environmental Health, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu, 807-8555, Japan.
authors.forename: Takatoshi
authors.initials: T
authors.lastname: Shimauchi
authors.forename: Kazunari
authors.initials: K
authors.lastname: Sugita
authors.forename: Motonobu
authors.initials: M
authors.lastname: Nakamura
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Tokura
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols; D001706:Biopsy; D017252:CD3 Complex / Q000032:analysis*; D015704:CD4 Antigens / Q000032:analysis*; D015496:CD4-Positive T-Lymphocytes / Q000187:drug effects / Q000276:immunology* / Q000473:pathology; D002478:Cells, Cultured; D003208:Concanavalin A / Q000494:pharmacology; D003873:Dermatitis, Exfoliative / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D005434:Flow Cytometry; D006801:Humans; D016130:Immunophenotyping; D016410:Lymphoma, T-Cell, Cutaneous / Q000150:complications / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D008297:Male; D008934:Mitogens / Q000494:pharmacology; D009360:Neoplastic Cells, Circulating / Q000276:immunology* / Q000473:pathology; D010641:Phenotype; D011948:Receptors, Antigen, T-Cell / Q000032:analysis; D054398:Receptors, CCR4 / Q000032:analysis; D012878:Skin Neoplasms / Q000150:complications / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D016896:Treatment Outcome
chemical_list: C515354:CCR4 protein, human; D017252:CD3 Complex; D015704:CD4 Antigens; D008934:Mitogens; D011948:Receptors, Antigen, T-Cell; D054398:Receptors, CCR4; D003208:Concanavalin A