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Pilomatricoma in Adult
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Pilomatricoma in Adult

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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

  • Epidermoid cyst can frequently be identified by observing an overlying punctum.
  • Pilar cyst is usually deeper than a pilomatricoma.
  • A pilomatricoma is typically firmer and less regular in shape than a dermoid cyst.
  • Glomus tumor is painful and tends to involve the nail unit.
  • Osteoma cutis presents as a firm dermal nodule and may be clinically indistinguishable from pilomatricoma.
  • Calcinosis cutis also presents as a firm dermal nodule and may be clinically indistinguishable from pilomatricoma.
  • Dermatofibroma

Best Tests

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Management Pearls

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Therapy

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References

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Last Updated: 05/08/2019
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Pilomatricoma in Adult
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Pilomatricoma : Blue color, Cyst, Face, Smooth nodule
Clinical image of Pilomatricoma
A deep red papule with overlying telangiectasias on the nose.
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