Rosai–Dorfman disease mimicking IgG4-related disease

Cetin O., Yetisir S., Akpinar T. S., Yegen G., Altinkaynak M.

Wiener Medizinische Wochenschrift, vol.176, no.1-2, pp.60-63, 2026 (ESCI, Scopus) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 176 Issue: 1-2
  • Publication Date: 2026
  • Doi Number: 10.1007/s10354-025-01110-x
  • Journal Name: Wiener Medizinische Wochenschrift
  • Journal Indexes: Emerging Sources Citation Index (ESCI), Scopus
  • Page Numbers: pp.60-63
  • Keywords: B symptoms, Histiocytic disorder, IgG4-related disease, Massive lymphadenopathy, Rosai–Dorfman disease
  • Istanbul University Affiliated: Yes

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.