Dahrouj Mohammad, Jakobiec Frederick A, Wolkow Natalie, Starks Victoria S, Lee Nahyoung Grace
Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA.
David G. Cogan Laboratory of Ophthalmic Pathology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts, USA.
Ocul Oncol Pathol. 2019 Feb;5(2):128-134. doi: 10.1159/000489127. Epub 2018 Jul 18.
BACKGROUND/AIMS: Rosai-Dorfman disease (RDD) is a rare, self-limited disorder of unknown etiology that affects children and young adults worldwide and typically manifests as chronic, painless cervical lymphadenopathy. Orbital involvement is very rare and may be an isolated extranodal manifestation or associated with concurrent systemic disease. Adjacent bone involvement is most exceptional, and secondary optic neuropathy has never been reported.
This is a case report with review of the literature.
We present a 32-year-old man who, over a 3-month period, developed worsening vision, headache, and vertical diplopia. On examination, there was decreased vision with dyschromatopsia, proptosis, and hypotropia of the left eye. CT scan of the orbits revealed a soft tissue mass inseparable from the lacrimal gland with adjacent bone erosion. Histopathologic evaluation revealed a diffuse infiltrate of histiocytes, lymphocytes, plasma cells, and neutrophils with peripolesis and emperipolesis (tunneling of lymphocytes and plasma cells in the histiocytes' cytoplasm without destruction), consistent with RDD. Resolution of symptoms as well as of the optic neuropathy was achieved with oral corticosteroids.
RDD is an important diagnosis that must be considered in the differential diagnosis of an orbital mass.
背景/目的：罗萨伊-多夫曼病（RDD）是一种病因不明的罕见自限性疾病，影响全球儿童和年轻人，通常表现为慢性无痛性颈部淋巴结病。眼眶受累非常罕见，可能是孤立的结外表现或与并发的全身疾病相关。相邻骨受累极为罕见，继发性视神经病变从未有过报道。
这是一篇病例报告并对文献进行了回顾。
我们报告一名32岁男性，在3个月内出现视力恶化、头痛和垂直性复视。检查发现视力下降伴色觉异常、眼球突出和左眼上斜视。眼眶CT扫描显示一个与泪腺无法区分的软组织肿块，伴有相邻骨质侵蚀。组织病理学评估显示组织细胞、淋巴细胞、浆细胞和中性粒细胞弥漫浸润，伴有嗜多色性和吞噬作用（淋巴细胞和浆细胞在组织细胞胞质内形成隧道而不被破坏），符合RDD。口服糖皮质激素后症状以及视神经病变得到缓解。
RDD是眼眶肿块鉴别诊断中必须考虑的重要诊断。
