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Pilomatricoma in Child
Other Resources UpToDate PubMed

Pilomatricoma in Child

Contributors: Craig N. Burkhart MD, Dean Morrell MD, Belinda Tan MD, PhD, Lowell A. Goldsmith MD, MPH
Other Resources UpToDate PubMed

Synopsis

Pilomatricoma, also known as calcifying epithelioma of Malherbe or pilomatrixoma, is a small benign tumor of hair cortex cell origin. A molecular defect in beta-catenin is present in the lesions. Pilomatricoma tends to arise during early childhood as solitary, asymptomatic tumors. Although usually sporadic in incidence, familial cases of pilomatricomas have been reported. Additionally, multiple pilomatricomas have been observed in patients with myotonic dystrophy, Gardner syndrome, Rubinstein-Taybi syndrome, constitutional mismatch repair deficiency, Kabuki syndrome, and trisomy 9, and in patients with PENS (papular epidermal nevus with "skyline" basal cell layer).

Codes

ICD10CM:
D23.9 – Other benign neoplasm of skin, unspecified

SNOMEDCT:
274901004 – Pilomatricoma

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Therapy

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References

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Last Updated:12/11/2019
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Pilomatricoma in Child
A medical illustration showing key findings of Pilomatricoma : Blue color, Cyst, Face, Smooth nodule
Clinical image of Pilomatricoma - imageId=179264. Click to open in gallery.  caption: 'A deep red papule with overlying telangiectasias on the nose.'
A deep red papule with overlying telangiectasias on the nose.
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