Sellari-Franceschini S, Lenzi R, Tognetti A, Seccia V
ENT Unit, Neuroscience Department of the University of Pisa, Italy.
Pathologica. 2010 Apr;102(2):62-6.
Rosai-Dorfman disease (RDD) is a rare benign condition of unknown origin, which was first described in 1969. By histopathology, the disease is composed of sinusoidal lymph node hyperplasia and abundant histiocytes with haemophagocytosis, particularly lymphocytes. It commonly affects lymph nodes, and rarely has an exclusively extra-nodal clinical presentation. Among the so-called "extranodal" sites, the head and neck region, and in particular the nose and paranasal sinuses, are frequently affected. RDD shows a highly variable clinical course that can be partly modified by medical therapy. We present of a case of extra-nodal RDD, with nasal and osseous involvement, which has been followed-up for 19 years. We also discuss its presentation, the most relevant radiographic findings, treatment options and histological findings.
罗萨伊-多夫曼病（RDD）是一种病因不明的罕见良性疾病，于1969年首次被描述。通过组织病理学检查，该病由窦状淋巴结增生和大量具有噬血细胞作用的组织细胞组成，尤其是淋巴细胞。它通常累及淋巴结，很少仅表现为结外临床表现。在所谓的“结外”部位中，头颈部区域，特别是鼻子和鼻窦，经常受到影响。RDD的临床病程高度可变，部分可通过药物治疗得到改善。我们报告一例累及鼻腔和骨质的结外RDD病例，该病例已随访19年。我们还讨论了其临床表现、最相关的影像学检查结果、治疗选择和组织学检查结果。
