Trichoepithelioma

Synonyms
Trichoepitheliom, Epithelioma adenoides cysticum, multiple familiäre Trichoepitheliome, desmoplastisches Trichoepitheliom, Brooke-Spiegler-Syndrom, multiple familial trichoepithelioma
Specialty
Dermatology · Skin tumours
Images
Clinical 2 · Histology 2
In the app
2 flashcards
Last updated
10/2026 · Dr. Pascal Bafteh
Contents
  1. Images (4)
  2. Definition
  3. Classification
  4. Aetiopathogenesis
  5. Clinical features
  6. Histology
  7. Diagnosis
  8. Differential diagnoses
  9. Keep learning in the app
  10. References (selection)
  11. Cross-references

Images (4)

Trichoepithelioma – face – clinical photo: skin-colored to pink, pearly nodule with telangiectasia on the nasal ala of a 39-year-old woman (BCC excluded histologically)
skin-colored to pink, pearly nodule with telangiectasia on the nasal ala of a 39-year-old woman (BCC excluded histologically) (face)Klaus D. Peter, Wiehl, Germany, „Trichoepithelioma", CC BY 3.0 de, via Wikimedia Commons · CC BY 3.0 de · Source
Trichoepithelioma – face – clinical photo: solitary dome-shaped pinkish skin-coloured papule with a small central crust on the cheek next to the ala of the nose
solitary dome-shaped pinkish skin-coloured papule with a small central crust on the cheek next to the ala of the nose (face)Klaus D. Peter, Wiehl, Germany, CC BY 3.0 DE, via Wikimedia Commons · CC BY 3.0 DE · Source
Trichoepithelioma – histology (H&E): monomorphic basaloid nests with several sharply outlined horn cysts in fibrous stroma, without retraction clefts towards the stroma; papillary mesenchymal bodies cannot …Histology
histology (H&E): monomorphic basaloid nests with several sharply outlined horn cysts in fibrous stroma, without retraction clefts towards the stroma; papillary mesenchymal bodies cannot …Nephron, „Trichoepithelioma - high mag", CC BY-SA 3.0, via Wikimedia Commons · CC BY-SA 3.0 · Source
Trichoepithelioma – histology (H&E), overview: well-circumscribed dermal basaloid tumour beneath an intact epidermis, lobules with peripheral palisading and retraction clefts towards the stroma, preserved …Histology
histology (H&E), overview: well-circumscribed dermal basaloid tumour beneath an intact epidermis, lobules with peripheral palisading and retraction clefts towards the stroma, preserved …Nephron, „Trichoepithelioma - 2 - intermed mag", CC BY-SA 3.0, via Wikimedia Commons · CC BY-SA 3.0 · Source
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Definition

Trichoepithelioma is a rare, benign adnexal tumour arising from the hair follicle. It occurs as a solitary lesion or as multiple lesions, mostly on the face.

Classification

  • Solitary trichoepithelioma
  • Multiple (familial) trichoepitheliomas
  • Desmoplastic trichoepithelioma: usually solitary, on the face

Aetiopathogenesis

Multiple familial trichoepitheliomas are inherited in an autosomal dominant manner and are due to germline mutations in the tumour suppressor gene CYLD. Brooke-Spiegler syndrome (trichoepitheliomas, cylindromas, spiradenomas) and familial cylindromatosis are now regarded as allelic variants of the same condition.

Clinical features

  • Firm, round, skin-coloured papules, mainly on the upper lip, nasolabial folds, nose, eyelids and forehead; less often on the scalp, neck or upper trunk.
  • Multiple trichoepitheliomas usually begin in early childhood or around puberty and are often symmetrical; solitary lesions often present in the second or third decade. Overall, trichoepitheliomas are most often diagnosed in adult women.
  • Desmoplastic trichoepithelioma is a ring-shaped lesion that can mimic basal cell carcinoma.

Histology

Dermal nests of basaloid cells with small horn cysts, abortive hair follicles and frequent calcification in a dense, cellular stroma; papillary mesenchymal bodies are a clue. Distinction from basal cell carcinoma can be difficult; retraction clefts between epithelium and stroma favour basal cell carcinoma.

Diagnosis

  • Provisional diagnosis from history and examination; dermoscopy: multiple white clods of variable size.
  • Definitive diagnosis is histological.
  • CYLD genetic testing for multiple trichoepitheliomas, or for a single tumour with an affected first-degree relative.

Differential diagnoses

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References (selection)

  1. DermNet: Trichoepithelioma
  2. DermNet: Trichoepithelioma pathology
  3. PubMed: CYLD GeneticTesting for Brooke-Spiegler Syndrome, Familial Cylindromatosis and Multiple Familial Trichoepitheliomas (PMID 25737804)
  4. PubMed: Mutations in the CYLD gene in Brooke-Spiegler syndrome, familial cylindromatosis, and multiple familial trichoepithelioma: lack of genotype-phenotype correlation (PMID 15854031)

Cross-references

Mentioned in

More skin tumours

Note: This page is intended for medical education and does not replace diagnosis or treatment decisions in individual cases.