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Rosai-Dorfman syndrome
Also known as:
Destombes-Rosai-Dorfman syndrome
Rosai-Dorfman Disease
Associated persons:
Pierre-Paul Louis Lucien Destombes
Ronald F. Dorfman
Juan Rosai
Description:
A disturbance that presents with massive painless lymphadenopathy in the neck, often bilateral. Extranodal involvement has been reported in about one third of the patients and may include the skin, lungs, bone, orbits, eyelids, kidneys, upper respiratory tract, peritoneum, salivary glands, testes and the central nervous system. The liver and spleen are not usually enlarged. Other features are fever, weight loss, tonsillitis and nasal discharge and obstruction. The condition is benign and there is usually spontaneous regression of symptoms with complete recovery.
We thank Bruno E. Paredes for information submitted.
Bibliography:
- P. P. L. Destombes:
Adenitis with lipid overload in infants and young adults, four observations in the Antilles and Mali.
Bulletin de La Société de Pathologie Exotique, 1965, 58: 1160-1171.
- J. Rosai, R. F. Dorfman:
Sinus histiocytis with massive lymphadenopathy. A newly recognized benign clinicopathological entity.
Archives of Pathology, Chicago, 1969, 87: 63-70.
- J. Rosai, R. F. Dorfman:
Sinus histiocytosis with massive lymphadenopathy. a pseudolymphomatous benign disorder. Analysis of 34 cases. Cancer 1972, 30: 1174-1188
- E. Foucar, J. Rosai, R. F. Dorfman:
Sinus histiocytis with massive lymphadenopathy; current status and future directions.
Archives of Dermatology, Chicago, 1988, 124: 1211-1214.
- E. Foucar, J. Rosai, R. Dorfman:
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Review of the entity.
Seminars in Diagnostic Pathology, 1990, 7: 19-73.
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