Pilomatricoma in Adult
Synopsis
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Additionally, multiple pilomatricomas have been observed in patients with myotonic dystrophy, familial adenomatous polyposis-related syndromes (including Gardner syndrome), Rubinstein-Taybi syndrome, constitutional mismatch repair deficiency, Kabuki syndrome, trisomy 9, Turner syndrome, and in patients with PENS (papular epidermal nevus with "skyline" basal cell layer). Patients with 6 or more pilomatricomas are more likely to have an underlying syndrome and should, therefore, undergo genetic screening.
Malignant transformation has been reported in fewer than 20 cases. Calcification, ossification, and epidermal perforation may occur.
Codes
D23.9 – Other benign neoplasm of skin, unspecified
SNOMEDCT:
274901004 – Pilomatrixoma
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Last Updated:07/08/2024
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