title: [NK/T-cell lymphoma, nasal type with cutaneous dissemination].
abstract: Extranodal NK/T-cell lymphoma, nasal type, is a lymphoproliferative disorder originating from peripheral T-cells or natural killer (NK) cells. While it is a rare disease in Europe, it is more frequent in Asia and South America. It is associated with Epstein-Barr virus (EBV) infection and characterized by an extremely aggressive course and poor prognosis. We report a 46-year-old Caucasian woman who presented with multiple subcutaneous, painful nodules on the trunk first noticed a few weeks earlier. In addition to dermatological findings, the patient reported a 4-months history of necrotizing nasopharyngeal inflammation of unclear origin. Due to nonspecific histological and clinical findings mimicking a chronic inflammatory condition, a diagnosis of Wegener disease was made and immunosuppressive therapy with azathioprine was initiated. However the disease progressed under therapy. Histopathological reevaluation and immunophenotyping revealed a disseminated NK/T-cell lymphoma, nasal type. In the case of an unspecific chronic inflammatory process in the nasopharyngeal space one should always consider the possibility of this rare lymphoma, even in Europe.
authors.affiliation: Hautklinik, Universitätsklinikums Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland.
authors.forename: A M
authors.initials: AM
authors.lastname: Baran
authors.forename: J
authors.initials: J
authors.lastname: Reifenberger
authors.forename: D
authors.initials: D
authors.lastname: Helbig
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D003520:Cyclophosphamide / Q000008:administration & dosage; D003937:Diagnosis, Differential; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D054391:Lymphoma, Extranodal NK-T-Cell / Q000188:drug therapy / Q000473:pathology*; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000188:drug therapy / Q000473:pathology*; D009669:Nose Neoplasms / Q000188:drug therapy / Q000473:pathology*; D011241:Prednisone / Q000008:administration & dosage; D012878:Skin Neoplasms / Q000188:drug therapy / Q000473:pathology*; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone