Spinal Rosai-Dorfman Disease with Isolated Severe Neuropathic Pain: A Case Report

Introduction: Rosia-Dorfman disease is a rare histiocytic disorder that presents as generalized lymphadenopathy. Rarely, patients experience atypical extra-nodal manifestations which can mimic other diseases. Case Presentation: We report a challenging diagnosis of Rosai-Dorfman disease in a 52-year-old female presenting with isolated symptomatic neuropathic pain. After excluding common diseases, a challenging diagnosis was made based on imaging, CSF studies and clinical progression that showed thoracic spine involvement and emperipolesis confirming RDD diagnosis and with a great clinical response to corticosteroids. Discussion: Clinical, laboratory and radiologic manifestations of the disease can vary a lot depending on the areas involved and patient progression. This can lead to challenges in establishing the diagnosis and deciding on treatment. Then unusual, first reported manifestations in our patient made the diagnosis exceptionally challenging, highlighting the importance of this report. Conclusion: This disease can have an atypical presentation that should be detected early to avoid any delay in diagnosis or treatment. Unusual cases must be reported to aid in such a difficult diagnosis in future similar cases.