World Library  
Flag as Inappropriate
Email this Article

Rosai–Dorfman disease

Article Id: WHEBN0014099112
Reproduction Date:

Title: Rosai–Dorfman disease  
Author: World Heritage Encyclopedia
Language: English
Subject: List of diseases (R), List of physicians, Non-Langerhans cell histiocytosis
Publisher: World Heritage Encyclopedia

Rosai–Dorfman disease

Rosai-Dorfman disease
Classification and external resources
Micrograph of a lymph node involved by Rosai-Dorfman disease showing emperipolesis. H&E stain.
ICD-10 D76.3 (ILDS D76.360)
DiseasesDB 31419

Rosai–Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare disorder of unknown etiology that is characterized by abundant histiocytes in the lymph nodes throughout the body.[1],[2]


Lymphadenopathy of the neck is the most common place of histiocyte accumulation, although accumulation outside of lymph nodes may occur, as well. The most common sites of accumulation outside of the lymph nodes are skin, upper respiratory tract, and the sinuses.[3][4] The disease can be also diagnosed in thyroid gland.[5]

The symptoms of this disease vary with the site of accumulation similar to other regional tumors. For instance, accumulation in closed spaces such as the cranium can lead to poor outcomes compared to growth in the dermis of an extremity where surgical excision is possible.

The disease is usually non-progressive and self-limited, but patients can manifest autoimmune hemolytic anemia. It can rarely present with massive hemoptysis. [6] Extra nodal sites such as skin, mucosae, spinal cord and pancreas may be involved.[1]


This condition has been named after Ronald F. Dorfman and Juan Rosai. An alternative eponym of this condition is known as Destombes-Rosai-Dorfman syndrome, part of which is named after Pierre-Paul Louis Lucien Destombes.

See also


  1. ^ a b Riyaz, Najeeba; Khader A (2005). "Rosai Dorfman Syndrome". Indian Journal of Dermatology, Venerology and Leprology 71 (5): 342–344. PMID 16394460. doi:10.4103/0378-6323.16786. Retrieved 2 December 2013. 
  2. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN . 
  3. ^ Foucar E, Rosai J, Dorfman R (1990). "Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity". Semin Diagn Pathol 7 (1): 19–73. PMID 2180012. 
  4. ^ Walid MS, Grigorian AA (2010). "Ethmo-spheno-intracranial Rosai-Dorfman disease". Indian J Cancer 47 (1): 80–81. PMID 20071801. doi:10.4103/0019-509X.58870. 
  5. ^ Lee FY, Jan YJ, Chou G, Wang J, Wang CC. (2007). "Thyroid involvement in Rosai-Dorfman disease.". Thyroid 17 (5): 471–6. PMID 17542678. doi:10.1089/thy.2006.0192. 
  6. ^ Journal, Chest; Lakshmi Varadarajalu, MD; Sunder Thirugnanasambandan, MS; Paul Jude Thangaraj, MS; Ashok Parameswaran, MD; Meghena Mathew*, MBBS (October 2012). "A Rare Presentation of Rosai Dorfman Disease". Chest. 4 142 (meeting abstracts): 998A–998A. doi:10.1378/chest.1389638. 

This article was sourced from Creative Commons Attribution-ShareAlike License; additional terms may apply. World Heritage Encyclopedia content is assembled from numerous content providers, Open Access Publishing, and in compliance with The Fair Access to Science and Technology Research Act (FASTR), Wikimedia Foundation, Inc., Public Library of Science, The Encyclopedia of Life, Open Book Publishers (OBP), PubMed, U.S. National Library of Medicine, National Center for Biotechnology Information, U.S. National Library of Medicine, National Institutes of Health (NIH), U.S. Department of Health & Human Services, and, which sources content from all federal, state, local, tribal, and territorial government publication portals (.gov, .mil, .edu). Funding for and content contributors is made possible from the U.S. Congress, E-Government Act of 2002.
Crowd sourced content that is contributed to World Heritage Encyclopedia is peer reviewed and edited by our editorial staff to ensure quality scholarly research articles.
By using this site, you agree to the Terms of Use and Privacy Policy. World Heritage Encyclopedia™ is a registered trademark of the World Public Library Association, a non-profit organization.

Copyright © World Library Foundation. All rights reserved. eBooks from World eBook Library are sponsored by the World Library Foundation,
a 501c(4) Member's Support Non-Profit Organization, and is NOT affiliated with any governmental agency or department.