Difference between revisions of "Juvenile xanthogranuloma"
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==General== | ==General== | ||
*Usually in children and infants, | *Usually in children and infants, sometimes in adults.<ref name=Ref_Derm622>{{Ref Derm|622}}</ref> | ||
*Most common form of non–Langerhans cell histiocytosis.<ref name=emed_jxg>URL: [http://emedicine.medscape.com/article/1111629-diagnosis http://emedicine.medscape.com/article/1111629-diagnosis]. Accessed on: 3 February 2011.</ref> | *Most common form of non–Langerhans cell histiocytosis.<ref name=emed_jxg>URL: [http://emedicine.medscape.com/article/1111629-diagnosis http://emedicine.medscape.com/article/1111629-diagnosis]. Accessed on: 3 February 2011.</ref> | ||
*Can rarely be found in the brain.<ref>URL: [http://path.upmc.edu/cases/case245/dx.html http://path.upmc.edu/cases/case245/dx.html]. Accessed on: 13 January 2012.</ref> | *Can rarely be found in the brain.<ref>URL: [http://path.upmc.edu/cases/case245/dx.html http://path.upmc.edu/cases/case245/dx.html]. Accessed on: 13 January 2012.</ref> | ||
Revision as of 16:10, 6 June 2013
Juvenile xanthogranuloma, abbreviated JXG, is a relatively common distinctive diagnosis in dermatopathology. It is also known as nevoxanthoendothelioma. In adults, it is called adult xanthogranuloma.[1]
General
- Usually in children and infants, sometimes in adults.[1]
- Most common form of non–Langerhans cell histiocytosis.[2]
- Can rarely be found in the brain.[3]
Microscopic
Features:[2]
- Dermal histiocytes:
- Abundant cytoplasm - may not be xanthomatous/foam cells.
- +/-Touton giant cell - key feature.
- Large multi-nucleated cells where nuclei are distributed at the cell periphery.
DDx:
- Langerhans cell histiocytosis.
- Spitz nevus - uncommon; reported to have Touton cells.[4]
- Dermatofibroma, aneurysmal - has Touton giant cells and hemosiderin deposition.
Notes:
- Must prove they are non-Langerhans cell histiocytes, esp. if no Touton giant cells.
Images
Juvenile xanthogranuloma - intermed. mag. (WC)
Touton giant cells in a juvenile xanthogranuloma - very high mag. (WC)
IHC
Features:[2]
- Langerhans cell markers: CD1a, CD207 -- both should be negative.
- If Touton giant cells are absent -- this is essential.
- Histiocyte markers: CD68, CD163 -- both should be positive.
- Vimentin +ve.
Other markers:[5]
- CD4 +ve (21 of 27 cases).
- CD45 +ve (25 of 27 cases).
- Factor XIIIa +ve (25 of 27 cases).
Negatives:[6]
- Muscle markers: actin, desmin.
- Others: S100, factor VIII, cytokeratins.
Sign out
SKIN LESION, CHIN, BIOPSY: - JUVENILE XANTHOGRANULOMA.
See also
References
- ↑ 1.0 1.1 Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 622. ISBN 978-0443066542.
- ↑ 2.0 2.1 2.2 URL: http://emedicine.medscape.com/article/1111629-diagnosis. Accessed on: 3 February 2011.
- ↑ URL: http://path.upmc.edu/cases/case245/dx.html. Accessed on: 13 January 2012.
- ↑ Guitart, J.; Gerami, P. (Jul 2008). "Touton-like giant cells in a Spitz's nevus.". J Cutan Pathol 35 (7): 694-5. doi:10.1111/j.1600-0560.2007.00877.x. PMID 18312437.
- ↑ Kraus, MD.; Haley, JC.; Ruiz, R.; Essary, L.; Moran, CA.; Fletcher, CD. (Apr 2001). "Juvenile xanthogranuloma: an immunophenotypic study with a reappraisal of histogenesis.". Am J Dermatopathol 23 (2): 104-11. PMID 11285404.
- ↑ Thomas DB, Sidler AK, Huston BM (October 1998). "Radiological case of the month. Juvenile xanthogranuloma". Arch Pediatr Adolesc Med 152 (10): 1029–30. PMID 9790615. http://archpedi.ama-assn.org/cgi/content/full/152/10/1029.