TrichoepitheliomaICD-11 Extension code

Trichoepithelioma is a benign tumour of hair follicle germ cell origin which typically presents in adults as a sporadic solitary cutaneous papule or nodule on the head or neck. Multiple trichoepitheliomata, typically appearing on the face, neck and upper trunk during adolescence, represent the commonest manifestation of the autosomal dominant hereditary hamartoneoplastic disorder, Brooke-Spiegler syndrome. Desmoplastic trichoepithelioma presents in young adults as a single slowly enlarging plaque containing hair follicle-like structures and may be confused both clinically and histologically with basal cell carcinoma.