Rosai Dorfman disease: Difference between revisions

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	Rosai-Dorfman disease
	Classification and external resources
ICD-10	D76.3 (ILDS D76.360)
DiseasesDB	31419

VisualWikitext

Latest revision as of 18:18, 26 September 2014

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2] Kiran Singh, M.D. [3]

Synonyms and keywords: Sinus histiocytosis with massive lymphadenopathy, SHML, Destombes-Rosai-Dorfman syndrome, familial Rosai–Dorfman disease, familial SHML, sinus histiocytosis

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

Micrograph of a lymph node involved by Rosai-Dorfman disease showing emperipolesis. H&E stain.

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.

Natural History, Complications and Prognosis

The symptoms of this disease vary with the site of accumulation similar to other regional tumors. For instance, accumulation in closed spaces such as the cranium can lead to poor outcomes compared to growth in the dermis of an extremity where surgical excision is possible.

Diagnosis

Symptoms

Fever

Physical Examination

Skin

Extremities

Rosai-dorfman disease. Adapted from Dermatology Atlas.^[4]
Rosai-dorfman disease. Adapted from Dermatology Atlas.^[4]
Rosai-dorfman disease. Adapted from Dermatology Atlas.^[4]
Rosai-dorfman disease. Adapted from Dermatology Atlas.^[4]
Rosai-dorfman disease. Adapted from Dermatology Atlas.^[4]
Rosai-dorfman disease. Adapted from Dermatology Atlas.^[4]
Rosai-dorfman disease. Adapted from Dermatology Atlas.^[4]
Rosai-dorfman disease. Adapted from Dermatology Atlas.^[4]
Rosai-dorfman disease. Adapted from Dermatology Atlas.^[4]
Rosai-dorfman disease. Adapted from Dermatology Atlas.^[4]

Head

Massive cervical lymph node enlargement

Ear

Sensorineural hearing loss may be present in some patients.

Abdomen

Hepatosplenomegaly

Laboratory Findings

References

↑ 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.
↑ ^4.0 ^4.1 ^4.2 ^4.3 ^4.4 ^4.5 ^4.6 ^4.7 ^4.8 ^4.9 "Dermatology Atlas".

Template:WH Template:WS

[Andrews-1] James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.

[pmid2180012-2] 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)

[3] 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.

[Dermatology_Atlas-4] 4.0 ^4.1 ^4.2 ^4.3 ^4.4 ^4.5 ^4.6 ^4.7 ^4.8 ^4.9 "Dermatology Atlas".

[1]

[2]

[3]

[4]

@@ Line 13: / Line 13: @@
 {{SI}}
-{{CMG}}; {{AE}} {{RT}}
+{{CMG}}; {{AE}} {{RT}} {{KS}}
-{{SK}} Sinus histiocytosis with massive lymphadenopathy, SHML, Destombes-Rosai-Dorfman syndrome, familial Rosai–Dorfman disease, familial SHML
+{{SK}} Sinus histiocytosis with massive lymphadenopathy, SHML, Destombes-Rosai-Dorfman syndrome, familial Rosai–Dorfman disease, familial SHML, sinus histiocytosis
 ==Overview==
@@ Line 21: / Line 21: @@
 ==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
+[[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.
+|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.
 ===Genetics===
@@ Line 42: / Line 42: @@
 * It is a disease of the childhood.
+==Natural History, Complications and Prognosis==
+The symptoms of this disease vary with the site of accumulation similar to other regional tumors. For instance, accumulation in closed spaces such as the [[cranium]] can lead to poor outcomes compared to growth in the dermis of an extremity where surgical excision is possible.
+==Diagnosis==
+===Symptoms===
+* [[Fever]]
+===Physical Examination===
+====Skin====
+=====Extremities=====
+<gallery>
+Image:Rosai-dorfman disease01.jpg|Rosai-dorfman disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Rosai-dorfman disease02.jpg|Rosai-dorfman disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Rosai-dorfman disease03.jpg|Rosai-dorfman disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Rosai-dorfman disease04.jpg|Rosai-dorfman disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Rosai-dorfman disease05.jpg|Rosai-dorfman disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Rosai-dorfman disease06.jpg|Rosai-dorfman disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Rosai-dorfman disease07.jpg|Rosai-dorfman disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Rosai-dorfman disease08.jpg|Rosai-dorfman disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Rosai-dorfman disease09.jpg|Rosai-dorfman disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+Image:Rosai-dorfman disease10.jpg|Rosai-dorfman disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  Adapted from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
+</gallery>
+====Head====
+* Massive cervical [[lymph node enlargement]]
+====Ear====
+* [[Sensorineural hearing loss]] may be present in some patients.
+====Abdomen====
+* [[Hepatosplenomegaly]]
+===Laboratory Findings===
+* [[Leukocytosis]]
+* [[Elevated erythrocyte sedimentation rate]] ([[ESR]])
+* [[Polyclonal hypergammaglobulinemia]]
-==Natural History, Complications and Prognosis==
+==References==
-The symptoms of this disease vary with the site of accumulation similar to other regional tumors. For instance, accumulation in closed spaces such as the cranium can lead to poor outcomes compared to growth in the dermis of an extremity where surgical excision is possible.
+{{reflist|2}}
+[[Category:Lymphoid-related cutaneous conditions]]
+[[Category:Rare diseases]]
+[[Category:Disease]]
+{{WH}}
+{{WS}}