The multiple basal-cell nevus syndrome in a Negro family

Abstract

Members of three generations of a Negro family with features of the multiple basal-cell nevus syndrome have been investigated. The proband, a 7-year-old boy was originally seen because of multiple odontogenic keratocysts. Further examination disclosed palmar pits and a bifid rib. His 28-year-old mother was found to have multiple jaw cysts, palmar and plantar pits, multiple nevi, and a calcifying ovarian fibroma. Her 30-year-old sister had multiple jaw cysts, palmar and plantar pits, basal-cell carcinoma and fibrosarcoma of the ovaries. The grandfather of the proband also had multiple jaw cysts and palmar pits plus calcification of the falx cerebri and bridging of the sella turcica. These findings support other authors' findings of a high degree of penetrance but a variable expressivity. Interestingly, only one of this kindred has had a malignant disease. The member is also the only one to have had a basal-cell carcinoma. It is because of the associated manifestations and management problems that this syndrome should be considered in any patient having multiple cysts of the jaws.