Pilomatricoma

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Pilomatricoma
File:Pilomatrixoma - high mag.jpg
Micrograph of a pilomatricoma showing the characteristic "ghost" cells (anucleate squamous cells), benign viable squamous cells and multi-nucleated giant cells. H&E stain.
Classification and external resources
Specialty Lua error in Module:Wikidata at line 446: attempt to index field 'wikibase' (a nil value).
ICD-10 D23
DiseasesDB 32128
eMedicine article/1058965
Patient UK Pilomatricoma
[[[d:Lua error in Module:Wikidata at line 863: attempt to index field 'wikibase' (a nil value).|edit on Wikidata]]]

Pilomatricoma, also known as a calcifying epithelioma of Malherbe,[1] Malherbe calcifying epithelioma, and Pilomatrixoma, is a benign skin tumor derived from the hair matrix.[2]:670[3]

Histologic features

Pilomatricomas consist of anucleate squamous cells (called "ghost cells"), benign viable squamous cells and multi-nucleated giant cells. The presence of calcifications is common.

Pathogenesis

Pilomatricoma is associated with high levels of beta-catenin caused by either a mutation in the APC gene or beta-catenin gene. These high levels of beta-catenin can aid cell proliferation, inhibit cell death, and ultimately lead to cancer.[4]

Associations

Pilomatricomas are associated with Turner syndrome, with a prevalence of 2.6%.[5] In addition to Turner syndrome, multiple pilomatricomas are associated with myotonic dystrophy, Rubinstein-Taybi syndrome, and trisomy 9.[6]

See also

References

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  4. Hassanein AM, Glanz SM, Kessler HP, Eskin TA, Liu C (2003). "beta-Catenin is expressed aberrantly in tumors expressing shadow cells. Pilomatricoma, craniopharyngioma, and calcifying odontogenic cyst". Am J Clin Pathol (5):732-6. PMID 14608900.
  5. Handler MZ, Derrick KM, Lutz RE, Morrell DS, Davenport ML, Armstrong AW (2013). "Prevalence of pilomatricoma in Turner syndrome: findings from a multicenter study". JAMA Dermatol 149(5):559-64. PMID 23426075.
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