Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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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PMID:Histiocytosis masquerading in the mesentery and pleura. 3179 91

Rosai-Dorfman disease (RDD) a rare, non-malignant disease of histiocytic proliferation usually presents as a painless lymphadenopathy. It has been associated with autoimmune diseases, viral infections, and malignancies. Management is the treatment of underlying disease. Here we present the case of a patient with HIV infection who presented with massive splenomegaly and lymphadenopathy.
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PMID:Rosai-Dorfman disease and superinfection due to Salmonella enterica and Mycobacterium avium complex in a patient living with HIV. 3222 66

Rosai-Dorfman disease (RDD) is a rare and self-limiting disease process that presents most commonly in young patients as massive, painless, cervical lymphadenopathy. Extranodal involvement may also occur. Histopathologic evaluation is the main diagnostic modality. We report an unusual presentation of RDD with cervical lymphadenopathy and an incidentally discovered sinonasal mass, clinically worrisome for malignancy. We emphasize that a high index of clinical suspicion is critical for accurate diagnosis of RDD. Clinicians and pathologists should consider RDD in a differential diagnosis of cervical lymphadenopathy, especially in young patients.
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PMID:Rosai-Dorfman Disease: A Rare Clinicopathological Presentation and Review of the Literature. 3250 87


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