Generalized dermal perifollicular fibromas with polyps of the colon

Hum Genet. 1976 Jul 27;33(2):193-7. doi: 10.1007/BF00281897.


The cases of 2 siblings with a rare, generalized genodermatosis have been reported, the most conspicuous feature being multiple perifollicular fibromas. In this type of dermal hamartomas, an apparent autosomal dominant trait was noted as well as the association with a small number of colon polyps in one of the patients. The author assumes a distinct dermo-intestinal syndrome not reported hitherto, which can be differentiated from Gardner's syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Female
  • Fibroma / genetics*
  • Humans
  • Intestinal Polyps / genetics*
  • Male
  • Middle Aged
  • Neoplasms, Multiple Primary / genetics*
  • Skin Neoplasms / genetics*
  • Syndrome