Familial external angular dermoid: evidence for a genetic link?

Congenital craniofacial dermoid cysts are hamartomas of ectodermal and mesodermal origin, made up of keratinizing epithelium lining a cavity containing dermal skin appendages. They usually present as an asymptomatic, firm, round mass, usually noticed at birth or in the first year. They are thought to occur as a result of ectodermal sequestration at lines of fusion during embryological development. Although nasal and epibulbar dermoid cysts have occurred in families and have been associated with inheritable gene defects, there has previously been no evidence for a similar etiology in external angular cases. We present the first description of external angular dermoids occurring in siblings suggesting that this subgroup may, in some instances, also have a genetic origin.