Brooke‐Spiegler Syndrome Associated with Cylindroma, Trichoepithelioma, Spiradenoma, and Syringoma

Abstract

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.