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Trichoepithelioma in Adult
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Trichoepithelioma in Adult

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Contributors: Daniel Gutierrez MD, Susan Burgin MD
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Synopsis

Trichoepitheliomas are benign neoplasms derived from the hair follicles. They usually present as asymptomatic smooth, skin-colored papules or nodules on the central face and scalp.

Various genetic syndromes can be associated with trichoepitheliomas. Mutations in CYLD on chromosome 9 are inherited in an autosomal-dominant pattern and can result in 3 distinct clinical phenotypes: Brooke-Spiegler syndrome, multiple familial trichoepitheliomas, and familial cylindromatosis. Both Brooke-Spiegler syndrome and multiple familial trichoepitheliomas are associated with development of multiple trichoepitheliomas in childhood or early adolescence. Because of decreased penetrance of this gene in men, such trichoepitheliomas are more commonly seen in female patients.

Desmoplastic trichoepithelioma (DTE) is a variant of trichoepithelioma that presents on the face as a slow-growing, skin-colored papule or plaque with a raised border and depressed center. DTEs are most commonly seen on the cheeks of young women. Solitary, multiple, familial, and nonfamilial presentations have been described. Inheritance patterns, however, are not well elucidated.

Related topic: Cylindroma

Codes

ICD10CM:
D23.30 – Other benign neoplasm of skin of unspecified part of face

SNOMEDCT:
274898000 – Trichoepithelioma

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

The differential diagnosis for trichoepitheliomas and DTEs includes the following:

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed: 10/15/2019
Last Updated: 11/01/2019
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Trichoepithelioma in Adult
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Trichoepithelioma : Cheek, Nose, Superior lip, Smooth papules, Smooth nodules
Clinical image of Trichoepithelioma
A large, smooth, shiny papule at the nasal alar crease.
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