Difference between revisions of "Rosai-Dorfman disease"
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| Line 12: | Line 12: | ||
| Molecular = | | Molecular = | ||
| IF = | | IF = | ||
| Gross = | | Gross = nodules (skin) | ||
| Grossing = | | Grossing = | ||
| Site = [[lymph node]]s - see ''[[lymph node pathology]]'' | | Site = [[skin]], [[lymph node]]s - see ''[[lymph node pathology]]'' | ||
| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = | ||
| Clinicalhx = | | Clinicalhx = | ||
| Signs = | | Signs = fever, lymphadenopathy | ||
| Symptoms = | | Symptoms = | ||
| Prevalence = rare | | Prevalence = rare | ||
| Bloodwork = | | Bloodwork = leukocytosis with neurophilia | ||
| Rads = | | Rads = | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = | | Prognosis = usually self-limited, benign | ||
| Other = | | Other = | ||
| ClinDDx = }} | | ClinDDx = lymphoma, infections with lymphadenopathy }} | ||
'''Rosai-Dorfman disease''', abbreviated '''RDD''', is a rare [[lymph node pathology]]. | '''Rosai-Dorfman disease''', abbreviated '''RDD''', is a rare [[lymph node pathology]]. | ||
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==General== | ==General== | ||
*Super rare. | *Super rare. | ||
*Prognosis - good.<ref name=pmid23739703/> | *Prognosis - good, usually self-limited.<ref name=pmid23739703/> | ||
Clinical findings:<ref name=pmid19668942>{{Cite journal | last1 = Landim | first1 = FM. | last2 = Rios | first2 = Hde O. | last3 = Costa | first3 = CO. | last4 = Feitosa | first4 = RG. | last5 = Rocha Filho | first5 = FD. | last6 = Costa | first6 = AA. | title = Cutaneous Rosai-Dorfman disease. | journal = An Bras Dermatol | volume = 84 | issue = 3 | pages = 275-8 | month = Jul | year = 2009 | doi = | PMID = 19668942 }}</ref><ref name=pmid23739703>{{Cite journal | last1 = Leal | first1 = PA. | last2 = Adriano | first2 = AL. | last3 = Breckenfeld | first3 = MP. | last4 = Costa | first4 = IS. | last5 = de Sousa | first5 = AR. | last6 = Gonçalves | first6 = Hde S. | title = Rosai-Dorfman disease presenting with extensive cutaneous manifestation - case report. | journal = An Bras Dermatol | volume = 88 | issue = 2 | pages = 256-9 | month = | year = | doi = 10.1590/S0365-05962013000200014 | PMID = 23739703 }}</ref> | Clinical findings:<ref name=pmid19668942>{{Cite journal | last1 = Landim | first1 = FM. | last2 = Rios | first2 = Hde O. | last3 = Costa | first3 = CO. | last4 = Feitosa | first4 = RG. | last5 = Rocha Filho | first5 = FD. | last6 = Costa | first6 = AA. | title = Cutaneous Rosai-Dorfman disease. | journal = An Bras Dermatol | volume = 84 | issue = 3 | pages = 275-8 | month = Jul | year = 2009 | doi = | PMID = 19668942 }}</ref><ref name=pmid23739703>{{Cite journal | last1 = Leal | first1 = PA. | last2 = Adriano | first2 = AL. | last3 = Breckenfeld | first3 = MP. | last4 = Costa | first4 = IS. | last5 = de Sousa | first5 = AR. | last6 = Gonçalves | first6 = Hde S. | title = Rosai-Dorfman disease presenting with extensive cutaneous manifestation - case report. | journal = An Bras Dermatol | volume = 88 | issue = 2 | pages = 256-9 | month = | year = | doi = 10.1590/S0365-05962013000200014 | PMID = 23739703 }}</ref> | ||
| Line 39: | Line 39: | ||
*Leukocytosis with neutrophilia. | *Leukocytosis with neutrophilia. | ||
*Polyclonal gammaglobulinemia. | *Polyclonal gammaglobulinemia. | ||
==Gross== | |||
*Skin: nodules.<ref name=pmid23739703/> | |||
==Microscopic== | ==Microscopic== | ||
Revision as of 21:15, 1 December 2013
| Rosai-Dorfman disease | |
|---|---|
| Diagnosis in short | |
 Rosai-Dorfman disease. H&E stain. | |
|
| |
| LM | sinus histiocytosis with histiocytes have a singular large round nucleus (~2x the size of a lymphocyte) with a prominent nucleolus (visible with 10x objective); emperipolesis |
| LM DDx | other specific histiocytoses (Langerhans cell histiocytosis, Erdheim-Chester disease), specific infections (rhinoscleroma, xanthogranulomatous pyelonephritis), sinus histiocytosis |
| IHC | CD68 -ve, S-100 +ve, CD1a -ve |
| Gross | nodules (skin) |
| Site | skin, lymph nodes - see lymph node pathology |
|
| |
| Signs | fever, lymphadenopathy |
| Prevalence | rare |
| Blood work | leukocytosis with neurophilia |
| Prognosis | usually self-limited, benign |
| Clin. DDx | lymphoma, infections with lymphadenopathy |
Rosai-Dorfman disease, abbreviated RDD, is a rare lymph node pathology.
It is also known as sinus histiocytosis with massive lymphadenopathy,[1] abbreviated SHML.
General
- Super rare.
- Prognosis - good, usually self-limited.[2]
- Fever.
- Leukocytosis with neutrophilia.
- Polyclonal gammaglobulinemia.
Gross
- Skin: nodules.[2]
Microscopic
Features:
- Sinus histiocytosis:
- Histiocytes - abundant.
- Singular large round nuclei[4] ~2x the size of resting lymphocyte.
- Prominent nucleolus - visible with 10x objective.
- Abundant cytoplasm.
- Singular large round nuclei[4] ~2x the size of resting lymphocyte.
- Histiocytes - abundant.
- Emperipolesis (from Greek: em = inside, peri = around, polemai = wander about[5]):
DDx:
- Other specific histiocytoses:
- Infection, e.g. rhinoscleroma (nasopharynx), xanthogranulomatous pyelonephritis.
- Sinus histiocytosis.
- Xanthomatous change.
Images
Emperipolesis in SHML (WC)
Rosai-Dorfman disease (WC)
Rosai-Dorfman disease - S-100 showing emperipolesis (WC)
www:
- RDD - case 1 - several images (upmc.edu).
- RDD - case 2 - several images of breast (upmc.edu).
- RDD - case 3 - several images (upmc.edu).
- RDD - case 4 - several images (upmc.edu).
IHC
- CD68 +ve.
- S-100 +ve.
- Useful for seeing emperipolesis.
- CD1a -ve.
- CD1a +ve in Langerhans cell histiocytosis.
See also
References
- ↑ Agarwal A, Pathak S, Gujral S (October 2006). "Sinus histiocytosis with massive lymphadenopathy--a review of seven cases". Indian J Pathol Microbiol 49 (4): 509–15. PMID 17183839.
- ↑ 2.0 2.1 2.2 Leal, PA.; Adriano, AL.; Breckenfeld, MP.; Costa, IS.; de Sousa, AR.; Gonçalves, Hde S.. "Rosai-Dorfman disease presenting with extensive cutaneous manifestation - case report.". An Bras Dermatol 88 (2): 256-9. doi:10.1590/S0365-05962013000200014. PMID 23739703.
- ↑ Landim, FM.; Rios, Hde O.; Costa, CO.; Feitosa, RG.; Rocha Filho, FD.; Costa, AA. (Jul 2009). "Cutaneous Rosai-Dorfman disease.". An Bras Dermatol 84 (3): 275-8. PMID 19668942.
- ↑ Bailey, D. 24 August 2010.
- ↑ Stedman's Medical Dictionary. 27th Ed.
- ↑ Viswanathan P, Raghunathan K, Majhi U, Pandit RV, Shanthi R, Rajkumar T (1997). Emperipolesis : an electron microscopic characteristic in RDD (Rosai-Dorfaman disease) : a case report. pp. 14-6. http://www.ijmpo.org/article.asp?issn=0971-5851;year=1997;volume=18;issue=1;spage=14;epage=16;aulast=Viswanathan;type=0.
- ↑ Lyons DJ, Gautam A, Clark J, et al. (January 1992). "Lymphocyte macrophage interactions: peripolesis of human alveolar macrophages". Eur. Respir. J. 5 (1): 59–66. PMID 1577151.