ArticleWiener medizinische Wochenschrift (1946)2026
Rosai-Dorfman disease mimicking IgG4-related disease.
Article in Wiener medizinische Wochenschrift (1946), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that may clinically and histologically resemble IgG4-related disease (IgG4-RD), especially in the presence of IgG4-positive plasma cell infiltration. In this case, a 69-year-old woman with generalized lymphadenopathy, constitutional symptoms, and elevated IgG4 levels was initially suspected to have IgG4-RD based on core needle biopsy. However, further evaluation with excisional lymph node biopsy revealed emperipolesis and S100-positive histiocytes, confirming the diagnosis of RDD. This case illustrates the diagnostic challenge of differentiating RDD from IgG4-RD, given the overlap in both clinical presentation and histopathological features, and highlights the importance of excisional biopsy combined with careful clinical evaluation to ensure an accurate diagnosis and appropriate management.
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