Rosai-Dorfman disease

Common Name(s)

Rosai-Dorfman disease

Rosai-Dorfman disease was first described by Rosai and Dorfman in 1969.  It is a benign disease which is characterized by over-production and accumulation of a specific type of white blood cell (histiocyte) in the lymph nodes of the body, most often those of the neck (cervical).  Other lymph node groups may also be involved and, in some cases, abnormal accumulation of histiocytes may occur in other areas of the body (extranodal). The cause of this condition remains unknown, although altered immune responses and infectious agents may play a role. SHML is a self-limited and seldom life-threatening disease which commonly does not require therapy.
 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Rosai-Dorfman disease" for support, advocacy or research.

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Histiocytosis Association

The Histiocytosis Association is dedicated to raising awareness about histiocytic disorders, providing educational and emotional support, and funding research leading to better treatments and a cure.

Last Updated: 30 Oct 2012

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Rosai-Dorfman disease" for support, advocacy or research.

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Histiocytosis Association

The Histiocytosis Association is dedicated to raising awareness about histiocytic disorders, providing educational and emotional support, and funding research leading to better treatments and a cure.

http://www.histio.org

Last Updated: 30 Oct 2012

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Rosai-Dorfman disease" returned 73 free, full-text research articles on human participants. First 3 results:

Case for diagnosis. Cutaneous Rosai-Dorfman disease.
 

Author(s): Han Ma, Huaiqiu Huang, Meirong Li, Wei Lai, Chun Lu

Journal: An Bras Dermatol. ;89(1):171-2.

 

Cutaneous Rosai-Dorfman disease is a rare, lymphoproliferative disease. It is benign and self-limited, only involves skin and subcutaneous tissue and typically occurs as histiocyte-rich inflammatory infiltrates, manifesting as erythematous to brown papules, plaques, or nodules, without ...

Last Updated: 14 Mar 2014

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Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease)--imaging manifestations of renal involvement.
 

Author(s): Sina Izadyar, Farhad Samiei, Ali Gholamrezanezhad

Journal: Nucl Med Rev Cent East Eur. 2014 ;17(1):44-6.

 

A 23-year-old lady presented with abdominal fullness and distension as well as large abdominal masses in physical exam. Upon ultrasonographic evaluation, two large space occupying lesions anterior to kidneys, with no clear distinction from renal tissue, as well as bilateral hydronephrosis ...

Last Updated: 10 Mar 2014

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Possible association of cutaneous Rosai-Dorfman disease and chronic Crohn disease: a case series report.
 

Author(s): Katrin A Salva, Melissa Stenstrom, Jonith Y Breadon, Paul Blair Odland, Daniel Bennett, Jack Longley, Gary S Wood

Journal: JAMA Dermatol. 2014 Feb;150(2):177-81.

 

IMPORTANCE Cutaneous Rosai-Dorfman disease (CRDD), a variant of Rosai-Dorfman disease limited to the skin, has a wide range of clinical presentations. Rosai-Dorfman disease is believed to result from an aberrant response to antigens, caused by immunosuppressive macrophages. Macrophage-mediated ...

Last Updated: 20 Feb 2014

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Rosai-Dorfman disease" returned 10 free, full-text review articles on human participants. First 3 results:

Generalized pure cutaneous Rosai-Dorfman disease: a link between inflammation and cancer not associated with mitochondrial DNA and SLC29A3 gene mutation?
 

Author(s): Min Zheng, Rui Bi, Wei Li, Lilla Landeck, Jia-Qi Chen, Li-Min Lao, Sui-Qing Cai, Yong-Gang Yao, Xiao-Yong Man

Journal: Discov Med. 2013 Nov;16(89):193-200.

 

Recently, we described a case of generalized pure cutaneous Rosai-Dorfman disease in a 43-year-old Asian man in JAMA. The lesions distributed on nearly all of the skin of the whole body, except for mucous sites. Molecular, immunophenotypic, and sequencing analyses seem to define it ...

Last Updated: 15 Nov 2013

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Extranodal Rosai-Dorfman disease involving appendix and mesenteric nodes with a protracted course: report of a rare case lacking relationship to IgG4-related disease and review of the literature.
 

Author(s): Ming Zhao, Changshui Li, Jiangjiang Zheng, Jingjing Yu, Hongcun Sha, Minghui Yan, Jie Jin, Ke Sun, Zhaoming Wang

Journal:

 

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare disease of unknown etiology that typically presents as nodal disease in young children. However, it also can present in various extranodal sites and can be difficult to recognize ...

Last Updated: 14 Nov 2013

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Intrathoracic Rosai-Dorfman disease with spontaneous remission: a clinical report and a review of the literature.
 

Author(s): Shingo Noguchi, Kazuhiro Yatera, Shohei Shimajiri, Naoyuki Inoue, Shuya Nagata, Chinatsu Nishida, Toshinori Kawanami, Hiroshi Ishimoto, Yasuyuki Sasaguri, Hiroshi Mukae

Journal: Tohoku J. Exp. Med.. 2012 ;227(3):231-325.

 

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-neoplastic disease that is characterized by a proliferation of histiocytes mostly in lymph nodes. However, the etiological mechanism of RDD still remains unclear. Intrathoracic ...

Last Updated: 13 Jul 2012

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

International Rare Histiocytic Disorders Registry (IRHDR)
 

Status: Recruiting

Condition Summary: Rare Histiocytic Disorders (RHDs); Juvenile Xanthogranuloma (JXG); Reticulohistiocytoma (Epithelioid Histiocytoma); Xanthoma Disseminatum (XD); Multicentric Reticulohistiocytosis (MRH); Systemic Juvenile Xanthogranuloma; Erdheim-Chester Disease (ECD); Multi-system Rosai-Dorfman Disease (RDD)

 

Last Updated: 4 Nov 2014

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