Brooke-Spiegler syndrome associated with cylindroma, trichoepithelioma, spiradenoma, and syringoma

J Dermatol. 2004 Jan;31(1):32-8. doi: 10.1111/j.1346-8138.2004.tb00501.x.


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.

Publication types

  • Case Reports

MeSH terms

  • Biopsy, Needle
  • Carcinoma, Adenoid Cystic / pathology*
  • Carcinoma, Adenoid Cystic / surgery
  • Carcinoma, Skin Appendage / pathology*
  • Carcinoma, Skin Appendage / surgery
  • Facial Neoplasms / pathology*
  • Facial Neoplasms / surgery
  • Female
  • Hair Follicle / pathology
  • Humans
  • Immunohistochemistry
  • Middle Aged
  • Prognosis
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / surgery
  • Sweat Gland Neoplasms / pathology*
  • Sweat Gland Neoplasms / surgery
  • Syndrome
  • Syringoma / pathology*
  • Syringoma / surgery