This is now recognized as a variant form of Brooke-Spiegler syndrome (spiradenoma, cylindroma, and trichoepithelioma
) caused by germline mutation in the CYLD gene.
is a benign neoplasm derived from follicular germ cells that presents as a skin-colored papule on the midface, especially the nose.
(2013) using BerEP4 to distinguish 24 BCC specimens with 88 common skin neoplasms, namely, trichoepithelioma
, actinic keratosis, squamous cell carcinoma in situ, squamous cell carcinoma, seborrheic keratosis, lichen planus like keratosis, nevi, hemangioma, inverted follicular keratosis, sebaceous adenoma, and Merkel cell carcinoma (MCC) from dermatopathology files, also showed that it was able to diffusely stain all BCC sections.
Krahl, "PHLDA1 (TDAG51) is a follicular stem cell marker and differentiates between morphoeic basal cell carcinoma and desmoplastic trichoepithelioma
," British Journal of Dermatology, vol.
Work comparing specific mutations in Brooke-Spiegler syndrome, familial cylindromatosis, and multiple familial trichoepithelioma
type 1 kindreds may reveal genotype-phenotype correlations and may promote increased understanding of disease mechanisms allowing for development of future therapeutic modalities .
TABLE Differential diagnosis for dermatomally distributed nodules (4) Neoplasms Benign Syringocystadenoma papilliferum, trichoepithelioma
, cutaneous schwannoma Malignant Basal cell carcinoma, cutaneous metastases, lymphoma, plasmacytoma, squamous cell carcinoma Other mucocutaneous Granuloma annulare, neurofibromatosis type 1, conditions pseudolymphoma, rheumatoid nodules, sarcoidosis, xanthomas Adapted with permission from: Hager CM, Cohen PR, Tschen JA.
Complex adnexal tumor of the primary epithelial germ with distinct patterns of superficial epithelioma with sebaceous differentiation, immature trichoepithelioma
, and apocrine adenocarcinoma.
is a benign adnexal tumour arising from hair matrix cells.
(9) Differential diagnosis between trichoepithelioma
(TE), trichoblastoma, trichofolliculoma, trichoadenoma, and BCC may be very difficult for the clinician and the pathologist.
CD10 Antibody (clone 56C6) preserves reactivity even in paraffin embedded material.5 In normal skin CD10 immunostainting is present in sebaceous glands, myoepithelial cells of eccrine and apocrine glands, periadnexal dermis, and inner root sheath cells of vellus hair follicles and occasional endothelial cells.6-8 CD10 has been recognized as a useful marker to differentiate BCC from trichoepithelioma
(TE).9,10 It is not routinely used to differentiate BCC from SCC but it has been claimed in some articles that CD10 can be utilized as a specific marker to distinguish BCC from SCC.4,11-13
It is of limited value in differentiating infiltrative BCC from desmoplastic trichoepithelioma
(DTE), although BCC shows diffuse staining and DTE shows partial staining 16.
(4) Marlowe and Cron reported a sebaceous nevus that transformed into three distinct neoplasms: an intradermal nevus, a trichoepithelioma
, and a basal cell carcinoma.