Extranodal Rosai–Dorfman disease manifesting as Sjögren’s syndrome combined with panuveitis and hypertrophic pachymeningitis: a case report and review of literature

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...

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Bibliographic Details
Published in:Journal of international medical research 2024-04, Vol.52 (4), p.3000605241233141-3000605241233141
Main Authors: Xu, Jing, Huang, Meihua, Dong, Binsong, Jian, Min, Chen, Jinyu, Zhang, Naiyuan, Ou, Chunlian, Wu, Yongming, Wang, Dongmei
Format: Article
Language:English
Subjects:
Adult
Case Report and Case Series
Female
Fluorodeoxyglucose F18
Histiocytosis, Sinus - complications
Histiocytosis, Sinus - diagnosis
Histiocytosis, Sinus - pathology
Humans
Hypertrophy
Panuveitis - complications
Panuveitis - diagnosis
Panuveitis - drug therapy
Positron Emission Tomography Computed Tomography
Sjogren's Syndrome - complications
Sjogren's Syndrome - diagnosis
Tomography
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Summary: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 18F-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.
ISSN:0300-0605
1473-2300
DOI:10.1177/03000605241233141