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Extranodal Rosai–Dorfman disease manifesting as Sjögren’s syndrome combined with panuveitis and hypertrophic pachymeningitis: a case report and review of literature
- Source :
- Journal of International Medical Research, Vol 52 (2024)
- Publication Year :
- 2024
- Publisher :
- SAGE Publishing, 2024.
-
Abstract
- Rosai–Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by massive lymphadenopathy and systemic extranodal lesions. We present the case of a 28-year-old woman who presented with recurrent blurred vision in her right eye for 3 months. She developed blindness and atrophy in her left eye a decade prior to presentation. She subsequently developed headache, fever, and impaired mental status. Cranial magnetic resonance imaging indicated hypertrophic pachymeningitis (HP), and 18 F-fluoro-2-deoxy-2- d -glucose (FDG) positron emission tomography/computed tomography revealed significant FDG uptake in the left dura mater. Autoimmune testing revealed elevated anti-nuclear, anti-SS-A, and anti-SS-B antibody levels. 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 for several weeks resulted in significant improvement. 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 a delayed diagnosis.
- Subjects :
- Medicine (General)
R5-920
Subjects
Details
- Language :
- English
- ISSN :
- 14732300 and 03000605
- Volume :
- 52
- Database :
- Directory of Open Access Journals
- Journal :
- Journal of International Medical Research
- Publication Type :
- Academic Journal
- Accession number :
- edsdoj.8c26d98a76514820bc0aba371d135a93
- Document Type :
- article
- Full Text :
- https://doi.org/10.1177/03000605241233141