title: CD30+ clonal T-cell lymphoid proliferation of the skin in a patient with hypereosinophilic syndrome.
abstract: We report a hitherto undescribed unusual CD30+ clonal T-cell proliferation in a 46-year-old man with the lymphocytic variant of hypereosinophilic syndrome with a 17-year history of pruritus, generalized persistent papulonodular skin lesions and peripheral blood hypereosinophilia. A skin biopsy showed an eosinophil-rich infiltrate with small to medium-sized CD30+ lymphocytes and Churg-Strauss granulomas. Peripheral blood flow cytometry revealed an aberrant T-cell clone which, molecular genetically, was identical to the T-cell clone detected in the skin. No genetic aberrations of platelet-derived growth factor receptor alpha (PDGFRA), FIP1L1-PDGFRA, PDGFRB or FGFR1 were found. The skin lesions showed transient response to systemic and topical corticosteroids. The skin lesions represent cutaneous involvement by clonal T-cells in hypereosinophilic syndrome and differ from known cutaneous CD30+ lymphoproliferative disorders.
authors.affiliation: Kempf und Pfaltz Histologische Diagnostik, Zurich, Switzerland.
authors.forename: Werner
authors.initials: W
authors.lastname: Kempf
authors.forename: Dmitry V
authors.initials: DV
authors.lastname: Kazakov
authors.forename: Zoltan
authors.initials: Z
authors.lastname: Szep
authors.forename: Tomas
authors.initials: T
authors.lastname: Vanecek
mesh_terms: D060965:Clonal Evolution; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000276:immunology* / Q000473:pathology; D017730:Ki-1 Antigen / Q000276:immunology*; D008213:Lymphocyte Activation; D016399:Lymphoma, T-Cell / Q000276:immunology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms / Q000276:immunology* / Q000473:pathology; D013601:T-Lymphocytes / Q000276:immunology* / Q000473:pathology
chemical_list: D017730:Ki-1 Antigen
keywords: CD30; anaplastic large cell lymphoma; hypereosinophilic syndrome; lymphomatoid papulosis