Rosai-Dorfman disease: an overview

Area of Science:

• Histiocytic Disorders
• Oncology
• Immunology

Background:

• Rosai-Dorfman disease is an uncommon histiocytic disorder often presenting as lymphadenopathy.
• Extranodal involvement is common, and the disease has been reclassified by the Histiocyte Society.
• Cutaneous Rosai-Dorfman disease is now considered a distinct entity.

Purpose of the Study:

• Review Rosai-Dorfman disease, including nodal, extranodal, and cutaneous forms.
• Highlight new insights into the potential clonal nature of the disease.
• Discuss the revised histiocytosis classification and the controversial association with IgG4-related disease.

Main Methods:

• Comprehensive review of published peer-reviewed English literature.

Main Results:

• Rosai-Dorfman disease and cutaneous Rosai-Dorfman disease are distinct entities per the revised classification.
• Increased IgG4-positive plasma cells can occur but require cautious interpretation.
• Molecular studies indicate a clonal process in a subset of Rosai-Dorfman disease cases.

Conclusions:

• The classification of Rosai-Dorfman disease is evolving with a better understanding of its pathogenesis.
• The potential neoplastic nature, suggested by clonality, warrants further investigation.
• The association with IgG4-related disease remains debated and requires careful consideration.