Rosai Dorfman disease
| Rosai-Dorfman disease | |
| Classification and external resources | |
| ICD-10 | D76.3 (ILDS D76.360) |
|---|---|
| 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.
Genetics
Rosai–Dorfman disease is inherited in an autosomal recessive pattern.
Microscopic Pathology
Differentiating from other diseases
The histological features of Rosai-Dorfman disease (RDD) resemble those of Faisalabad histiocytosis. Faisalabad histiocytosis can be differentiated from RDD by the presence of joint problems, sensorineural hearing loss and rubbery swellings over the eyelids.
Epidemiology and Demographics
- The incidence of this disease is <10/100 000.
- It is a disease of the childhood.
- ↑ 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.
|access-date=requires|url=(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.