trichoepithelioma

Trichoepithelioma is a neoplasm of the adnexa of the skin.

A desmoplastic trichoepithelioma is a cutaneous condition characterized by a solitary, firm skin lesion on the face.

A giant solitary trichoepithelioma is a cutaneous condition characterized by a skin lesion that may be up to several centimeters in diameter.

Desmoplastic trichoepithelioma is benign tumor and can be managed safely with surgical removal, electrodesiccation and curettage.

Other skin lesions that may need to be considered include cylindroma, lipoma, poroma and trichoepithelioma; these tend to be painless and have other useful distinguishing features.

Harada H, Hashimoto K, Ko MS: The gene for multiple familial trichoepithelioma maps to chromosome 9p21.

Three conditions are known to be caused by mutations in the CYLD gene: Brooke-Spiegler syndrome, multiple familial trichoepithelioma and familial cylindromatosis.

Multiple familial trichoepithelioma (also known as "Brooke-Spiegler syndrome" and "Epithelioma adenoides cysticum") is a cutaneous condition characterized by multiple cystic and solid nodules appearing on the face.

Brooke-Spiegler Syndrome (BSS), familial cylindromatosis, and multiple familial trichoepithelioma (MFT) are all autosomal dominant syndromes with overlapping clinical characteristics with allelic variance.

Young AL, Kellermayer R, Szigeti R, et al.: CYLD mutations underlie Brooke-Spiegler, familial cylindromatosis, and multiple familial trichoepithelioma syndromes.

Bowen S, Gill M, Lee DA, et al.: Mutations in the CYLD gene in Brooke-Spiegler syndrome, familial cylindromatosis, and multiple familial trichoepithelioma: lack of genotype-phenotype correlation.