We present an atypical presentation of Rosai-Dorfman disease (RDD). Due to its overlap with IgG4-related disease (IgG4-RD), this case proved to be a diagnostic dilemma. Our case is an example of the importance of having a broad-based differential and, ultimately, an in-depth histopathological review. Our patient presented with a constellation of symptoms suggestive of an underlying malignancy. He was provisionally diagnosed with peritoneal carcinomatosis of an unknown primary. His initial presentation triggered a series of investigations, surgery and biopsies. Omental biopsy specimens were suggestive of IgG4-RD. Despite appropriate treatment for IgG4-RD, his disease progressed, specifically in the lungs. Pleural biopsies were then collected and assessed alongside the omental biopsies. On review and reassessment, the patient was formally diagnosed with RDD.
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Contributors All authors provided substantial intellectual contribution and participation in the generation of this case report. ND, was responsible for the the acquisition/collection of all information and the chronological assessment of all information; she wrote the original draft, made the revisions decided upon by the group and finalised all edits. SD also contributed substantially to the write-up, research and references involved in this article generation. MC was the physician in charge of taking care of the patient. She provided guidance on the timeline for the patient’s case, along with improvements to the original draft and editing of the multiple draft versions that were created by ND and SD. MC helped research the case and contributed to the discussion and writing of the case. Ultimately, she signed off on the case report when she felt it was ready to be submitted for consideration to the BMJ Case Reports. Her expertise in the recognition and treatment of Rosai-Dorfman disease were pivotal in navigating the approach to this case report, finding resources and helping develop a succintly written case report. BS was instrumental in getting the final diagnosis correct. He provided thorough histopathological analysis and interpretation of the histopathological specimens, and ultimately helped guide the treating team to the correct diagnosis.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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