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Pilomatrixoma

ORPHA91414
Synonym(s) Epithelioma calcificans of Malherbe
Pilomatricoma
Prevalence Unknown
Inheritance Not applicable
Age of onset Childhood
Adolescent
Adult
ICD-10
  • C44.3
  • C44.6
OMIM
UMLS
  • C0206711
MeSH
  • D018296
MedDRA
  • 10035040

Summary

Pilomatrixoma is a rare benign hair cell-derived tumor occurring mostly in young adults (usually under the age of 20) and characterized as a 3 to 30 mm solitary, painless, firm, mobile, deep dermal or subcutaneous tumor, most commonly found in the head, neck or upper extremities. When superficial, the tumors tint the skin blue-red. Multiple pilomatrixomas are seen in myotonic dystrophy, Gardner syndrome, Rubinstein-Taybi syndrome, and Turner syndrome (see these terms).


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