Autosomal dominant familial frontonasal dermoid cysts: a mother and her identical twin daughters.

ABSTRACT

A dermoid cyst is an ectodermal cyst that contains an epithelial lining as well as adnexal structures, and may occur in numerous areas of the body. The nasal dermoid accounts for 1% of all dermoid cysts and 3-12% of head and neck dermoid cysts. While there have been familial cases reported, a genetic inheritance for nasal dermoids has not been suggested. We present the first reported case of a mother and her identical twin daughters who were all found to have evidence of frontonasal dermoid cysts. Our case and a review of literature seem to implicate an autosomal dominant inheritance in certain instances of nasal dermoids.