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Pilomatricoma - Skin in Infant/Neonate
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Pilomatricoma - Skin in Infant/Neonate

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Contributors: Belinda Tan MD, PhD, Craig N. Burkhart MD, Dean Morrell MD, Lowell A. Goldsmith MD, MPH, Nancy Esterly MD
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Synopsis

Pilomatricoma, sometimes called Malherbe's calcifying epithelioma or pilomatrixoma, are benign, small tumors of hair cortex cell origin. A molecular defect in beta-catenin is present in the lesions. Pilomatricoma have been reported in infants but tend to arise during early childhood, usually before puberty, 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's syndrome, and Rubinstein-Taybi syndrome.

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: 12/13/2013
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Pilomatricoma - Skin in Infant/Neonate
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Pilomatricoma : Blue color, Cyst, Face, Smooth nodule
Clinical image of Pilomatricoma
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