Birt-Hogg-Dubé syndrome: a review and presentation of the first case with oral lesions

Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1997 Apr;83(4):496-500. doi: 10.1016/s1079-2104(97)90152-9.


Birt-Hogg-Dubé syndrome manifests as small, skin-colored, dome-shaped, or pedunculated cutaneous lesions. Three histopathologic patterns have been described: fibrofolliculomas, trichodiscomas, and acrochordons. The patient described in this article also had small, elevated, intraoral papules involving the mucosal surfaces of the lips, buccal mucosa, and gingivae, which represents a finding that had not been described in the literature previously.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Fibroma / pathology
  • Gingival Neoplasms / pathology*
  • Hair Follicle / pathology
  • Head and Neck Neoplasms / pathology*
  • Humans
  • Male
  • Middle Aged
  • Mouth Mucosa / pathology*
  • Neoplasms, Basal Cell / pathology*
  • Skin Neoplasms / pathology*
  • Syndrome