Pilomatrixoma

on 19.7.06 with 0 comments



Synonyms: Pilomatricoma, calcifying epithelioma of Malherbe
  • •Peak age 8-13 years
  • •Female > male
  • •70% on face & neck
  • •3.5% multiple
  • Adnexal tumor differentiating toward hair matrix
  • –Internal root sheath
  • –Hair shaft
•Histology
  1. –Early: cystic
  2. –Late: bluish hard nodule, chalky white on cut section
  3. –Blue: basaloid matrical cells, Pink: ghost/shadow cells
  4. –Osseous metaplasia
  5. –Mitoses common in matrical cells
Disease associations
  • –Gardner’s
  • –Myotonic dystrophy
  • •
  1. Autosomal dominant
  2. •
  3. Myotonia, muscle wasting, cardiac conduction defects, cataracts, testicular atrophy, frontal baldness

    Variations:
  • giant form, proliferating, malignant
  • Beta-catenin mutation
  • Shadow cells are non-specific
  • •Desmoplastic trichoepithelioma
  • •Mixed tumor
  • •Matrical carcinoma

Category: Dermatology Notes

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