Extranodal Rosai-Dorfman Disease Manifesting as Sjögren’s Syndrome Combined with Panuveitis and Hypertrophic Pachymeningitis: A Case Report and Review of Literature
Research Square (Research Square)(2023)
摘要
Abstract Background: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by massive lymphadenopathy and systemic extranodal lesions. Case presentation: We present a case of 28-year-old female who presented with recurrent blurred vision in her right eye for 3 months. Her left eye had been blinded and atrophied a decade prior. She subsequently developed a headache, fever, and impaired mental status. Cranial MRI indicated hypertrophic pachymeningitis (HP), and 18 F-FDG PET/CT revealed significant FDG uptake in the left dura mater. Autoimmune testing revealed elevated anti-nuclear, anti-SS-A, and anti-SS-B antibodies. Incisional biopsy of the atrophic eyeball revealed RDD with marked polyclonal plasmacytosis. The patient was diagnosed with RDD accompanied by multisystem involvement, including Sjögren’s syndrome (SS), panuveitis, and HP. Treatment with methylprednisolone over several weeks resulted in significant improvement. Conclusions: This is the first reported case of RDD presenting with SS in combination with panuveitis and HP. Although RDD is rarely diagnosed in young patients, interdisciplinary collaboration is essential to prevent delayed diagnosis.
更多查看译文
关键词
sjögrens,panuveitis,hypertrophic pachymeningitis,disease,rosai-dorfman
AI 理解论文
溯源树
样例
生成溯源树,研究论文发展脉络
Chat Paper
正在生成论文摘要