Olmsted syndrome--a rare syndrome with oral manifestations

Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2004 May;97(5):599-602. doi: 10.1016/S1079210403006619.


Olmsted syndrome is a rare, congenital condition characterized by severe palmo-planter keratosis, periorificial keratosis, and hypotrichosis. Though orofacial keratosis is one of the consistent findings of Olmsted syndrome, it has never been reported in the dental literature. We report a case of Olmsted syndrome in an eight-year-old boy who presented with massive and crippling palmoplanter keratosis and bilateral oral lesions in the form of keratotic plaques at the corners of the mouth, as well as on the dorsum of the tongue.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Facial Dermatoses / congenital
  • Humans
  • Hypotrichosis / congenital*
  • Keratoderma, Palmoplantar / congenital*
  • Keratosis / congenital*
  • Leukoplakia, Oral / congenital
  • Lip Diseases / congenital*
  • Male
  • Syndrome
  • Tongue Diseases / congenital