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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
- –Early: cystic
- –Late: bluish hard nodule, chalky white on cut section
- –Blue: basaloid matrical cells, Pink: ghost/shadow cells
- –Osseous metaplasia
- –Mitoses common in matrical cells
Disease associations - –Gardner’s
- –Myotonic dystrophy
- •
- Autosomal dominant
- •
- 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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