Brooke-Spiegler syndrome associated with cylindroma, trichoepithelioma, spiradenoma, and syringoma.
Brooke-Spiegler syndrome is a rare, autosomally dominant disease characterized by the development of multiple cylindromas, trichoepitheliomas, and occasional spiradenomas. We report herein a case of Brooke-Spiegler syndrome in a 55-year-old woman associated with multiple cylindromas, spiradenomas, trichoepitheliomas, and syringomas that developed on her face. Her 52-year-old sister also suffered from multiple trichoepitheliomas. Although there is variable clinical and histological expression in Brooke-Spiegler syndrome, all of the tumors encountered in our case were related to folliculosebaceous-apocrine units.
Uede
Koji
K
Department of Dermatology, Wakayama Medical University, Wakayama, Japan.
Yamamoto
Yuki
Y
Furukawa
Fukumi
F
D001707:Biopsy, Needle; D003528:Carcinoma, Adenoid Cystic / Q000473:pathology* / Q000601:surgery; D018280:Carcinoma, Skin Appendage / Q000473:pathology* / Q000601:surgery; D005153:Facial Neoplasms / Q000473:pathology* / Q000601:surgery; D005260:Female; D018859:Hair Follicle / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008875:Middle Aged; D011379:Prognosis; D012878:Skin Neoplasms / Q000473:pathology* / Q000601:surgery; D013544:Sweat Gland Neoplasms / Q000473:pathology* / Q000601:surgery; D013577:Syndrome; D018252:Syringoma / Q000473:pathology* / Q000601:surgery