Hereditary benign intraepithelial dyskeratosis: Report of two cases with prominent oral lesions

J Am Acad Dermatol. 2001 Oct;45(4):634-6. doi: 10.1067/mjd.2001.116336.


Hereditary benign intraepithelial dyskeratosis is a rare autosomal dominant disorder of the oral and ocular mucosa initially described in the Haliwa-Saponi Native American tribe of North Carolina. We describe 2 sisters with the characteristic oral and ocular findings. This entity should be distinguished from several other diseases that cause white lesions in the mouth including white sponge nevus.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Conjunctiva / pathology*
  • Diagnosis, Differential
  • Dyskeratosis Congenita / genetics
  • Dyskeratosis Congenita / pathology*
  • Female
  • Humans
  • Mouth Mucosa / pathology*