Rosai–Dorfman disease mimicking IgG4-related disease

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

Wiener Medizinische Wochenschrift, 2025 (ESCI) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Basım Tarihi: 2025
  • Doi Numarası: 10.1007/s10354-025-01110-x
  • Dergi Adı: Wiener Medizinische Wochenschrift
  • Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), Scopus
  • Anahtar Kelimeler: B symptoms, Histiocytic disorder, IgG4-related disease, Massive lymphadenopathy, Rosai–Dorfman disease
  • İstanbul Üniversitesi Adresli: Evet

Özet

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.