Intravascular large B-cell lymphoma (IVLBL) is a rare type of non-Hodgkin’s lymphoma characterized by selective growth of neoplastic cells within the vascular

© 2010 The Authors. doi: 10.2340/00015555-0817 Journal Compilation © 2010 Acta Dermato-Venereologica. ISSN 0001-5555 Intravascular large B-cell lymphoma (IVLBL) is a rare type of non-Hodgkin’s lymphoma characterized by selective growth of neoplastic cells within the vascular lumen (1). IVLBL frequently manifests with cutaneous symptoms of various morphologies and distributions, such as painful indurated erythematous eruption, poorly circumscribed violaceous plaques, cellulitis, large solitary plaques, painful blue-red palpable nodular discolorations, tumours, ulcerated nodules, small red palpable spots, or erythematous desquamative plaques (2). Although IVLBL is generally regarded as a disseminated disease (1), “cutaneous variants” of IVLBL exist; these are limited exclusively to the skin and are classified as primary cutaneous IVLBL by the World Health Organization– European Organization for Research and Treatment of Cancer (WHO–EORTC) (2, 3). IVLBL is associated with poor prognosis in most patients, but patients with cutaneous variants have better survival (3). Although anthracycline-based chemotherapy has been the cornerstone of IVLBL treatment (4), no standard treatment guidelines have yet been established (5). Recently, rituximab-containing chemotherapies have appeared promising (4, 6, 7), but neither the efficacy nor safety of such chemotherapies has been sufficiently evaluated, especially in treatment of cutaneous variants.