Rosai Dorfman disease: Difference between revisions
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==Historical Perspective== | ==Historical Perspective== | ||
This condition has been named after Ronald F. Dorfman and Juan Rosai. The alternative eponym of this condition is known as Destombes-Rosai-Dorfman syndrome, part of which is named after Pierre-Paul Louis Lucien Destombes. | This condition has been named after Ronald F. Dorfman and Juan Rosai. The alternative eponym of this condition is known as Destombes-Rosai-Dorfman syndrome, part of which is named after Pierre-Paul Louis Lucien Destombes. | ||
==Pathophysiology== | |||
[[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.<ref name="pmid2180012">{{cite journal |author=Foucar E, Rosai J, Dorfman R |title=Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity | |||
|journal=Semin Diagn Pathol |volume=7 |issue=1 |pages=19–73 |year=1990 | |||
|pmid=2180012 |doi= |accessdate=2007-11-05}}</ref><ref>{{cite journal | |||
|author=Walid MS, Grigorian AA |title=Ethmo-spheno-intracranial Rosai-Dorfman disease |journal=Indian J Cancer |volume=47 |issue=1 |pages=80–81 |year=2010 |pmid= 20071801 |doi=10.4103/0019-509X.58870}}</ref> In approximately 25% of the situations [[salivary gland]]s, [[spleen]], liver and eyelids can be involved in the disease process. |
Revision as of 19:18, 31 July 2012
Rosai-Dorfman disease | |
Classification and external resources | |
ICD-10 | D76.3 (ILDS D76.360) |
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DiseasesDB | 31419 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]
Synonyms and keywords: Sinus histiocytosis with massive lymphadenopathy, SHML, Destombes-Rosai-Dorfman syndrome, familial Rosai–Dorfman disease, familial SHML
Overview
Rosai–Dorfman disease is a rare disorder of unknown etiology that is characterized by abundant histiocytes in the lymph nodes throughout the body.[1]:747
Historical Perspective
This condition has been named after Ronald F. Dorfman and Juan Rosai. The alternative eponym of this condition is known as Destombes-Rosai-Dorfman syndrome, part of which is named after Pierre-Paul Louis Lucien Destombes.
Pathophysiology
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.[2][3] In approximately 25% of the situations salivary glands, spleen, liver and eyelids can be involved in the disease process.
- ↑ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
- ↑ 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.
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(help) - ↑ Walid MS, Grigorian AA (2010). "Ethmo-spheno-intracranial Rosai-Dorfman disease". Indian J Cancer. 47 (1): 80–81. doi:10.4103/0019-509X.58870. PMID 20071801.