Mudhar Hardeep Singh, Duke Roseline
National Specialist Ophthalmic Pathology Service, Dept of Histopathology, Royal Hallamshire Hosiptal, Sheffield , England , United Kingdom , and.
Orbit. 2013 Oct;32(5):315-7. doi: 10.3109/01676830.2013.799705. Epub 2013 Jul 22.
A case of a 9-year-old Black African male with right-sided proptosis is presented. The clinical interpretation was that of orbital rhabdomyosarcoma and an incisional biopsy was performed. This revealed the typical features of Rosai-Dorfman disease, featuring S100 positive non-Langerhans histiocytes with emperipolesis. There were also numerous plasma cells in the biopsy, in the more fibrotic areas. More than 40% of these plasma cells were positive for IgG4. This case represents the first case in the orbit to make the association of IgG4 plasma cell positivity with Rosai-Dorfman disease. Potential associations are discussed, and the relevance of this finding to ophthalmic pathology interpretation and differential diagnosis are highighted.
本文报告一例9岁非洲裔黑人男性右侧眼球突出病例。临床诊断为眼眶横纹肌肉瘤，并进行了切开活检。结果显示为Rosai-Dorfman病的典型特征，即S100阳性的非朗格汉斯组织细胞伴细胞内吞噬现象。活检中在纤维化程度较高的区域也有大量浆细胞。这些浆细胞中超过40%的IgG4呈阳性。该病例是眼眶中首例将IgG4浆细胞阳性与Rosai-Dorfman病相关联的病例。文中讨论了可能的关联，并强调了这一发现对眼科病理诊断和鉴别诊断的相关性。
