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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Rosai-Dorfman disease


Other Names for this Disease

  • RDD
  • Rosaï-Dorfman disease
  • SHML
  • Sinus histiocytosis with massive lymphadenopathy
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Symptoms

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What are the signs and symptoms of Rosai-Dorfman disease?

The symptoms and physical findings associated with Rosai-Dorfman disease vary depending upon the specific areas of the body that are affected.[1] Systemic symptoms, such as fever, weight loss, malaise, joint pain, and night sweats, may be present. Cervical lymph nodes are most characteristically involved, but other areas, including extranodal regions, can be affected.[2] These areas include the skin, central nervous system, kidney, and digestive tract.[1] 

Last updated: 7/22/2013

The Human Phenotype Ontology provides the following list of signs and symptoms for Rosai-Dorfman disease. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Abnormal immunoglobulin level 90%
Abnormality of temperature regulation 90%
Anemia 90%
Lymphadenopathy 90%
Muscle weakness 90%
Abnormality of the skin 7.5%

Last updated: 11/3/2014

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.


References
  1. Rosai J. Rosai-Dorfman Disease. National Organization for Rare Disorders (NORD). 2011; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1122/viewAbstract. Accessed 7/22/2013.
  2. Tebbi CK, Arceci RJ, Loew TW. Histiocytosis. Medscape Reference. April 2012; http://emedicine.medscape.com/article/958026-overview. Accessed 7/22/2013.


Other Names for this Disease
  • RDD
  • Rosaï-Dorfman disease
  • SHML
  • Sinus histiocytosis with massive lymphadenopathy
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.