Article Text

Download PDFPDF
Rosai-Dorfman disease: an overview
  1. Cassandra Bruce-Brand1,2,
  2. Johann W Schneider1,2,
  3. Pawel Schubert1,2
  1. 1Stellenbosch University, Faculty of Medicine and Health Sciences, Division of Anatomical Pathology, Cape Town, South Africa
  2. 2National Health Laboratory Service, Tygerberg Hospital, Cape Town, South Africa
  1. Correspondence to Dr Cassandra Bruce-Brand, Anatomical Pathology, Stellenbosch University, PO Box 241, Cape Town, 8000, South Africa; cassandra.bruce-bran{at}


Context Rosai-Dorfman disease is an uncommon histiocytic disorder most frequently presenting as bilateral cervical lymphadenopathy in children and young adults. Extranodal disease occurs in a significant proportion of patients. It has been recently classified as part of the ‘R group’ of histiocytoses by the Histiocyte Society in 2016. Cutaneous Rosai-Dorfman disease is regarded as a separate disease entity that falls into the ‘C group’ of histiocytoses according to this classification system. The pathogenesis was previously poorly understood; however, recent evidence demonstrating clonality in a subset of cases raises the possibility of a neoplastic process. A possible association with IgG4-related disease remains controversial.

Objectives To provide a comprehensive review of Rosai-Dorfman disease, including nodal, extranodal and cutaneous forms, with a particular emphasis on new insights into the possible clonal nature of the disease; to discuss the recently revised classification of the histiocytoses by the Histiocyte Society; and to summarise the findings from the literature regarding the controversial association with IgG4-related disease.

Data sources This review is based on published peer-reviewed English literature.

Conclusions Classic Rosai-Dorfman disease, which may be sporadic or familial, is considered a separate entity from cutaneous disease, which is reflected in the revised classification of histiocytoses. An increase in IgG4-positive plasma cells may be seen in Rosai-Dorfman disease. This finding in isolation is of limited significance and should be interpreted with caution. Studies investigating the molecular profile of the disease show that in at least a subset of cases the disease is a clonal process. The classification of Rosai-Dorfman disease is therefore likely to change as our understanding of the aetiopathogenesis evolves.

  • haematological disease
  • pathology, surgical
  • lymph nodes
View Full Text

Statistics from


  • Handling editor Runjan Chetty.

  • Contributors All authors have contributed to the production of this manuscript.

  • 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 Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.