Extranodal Rosai-Dorfman Disease Manifesting as Sjögren’s Syndrome Combined with Panuveitis and Hypertrophic Pachymeningitis: A Case Report and Review of Literature

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 18F-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.