[Gorlin-Gotz syndrome in 3 patients from the same family monitored from 1993 to 1999].
The authors describe the clinical symptoms in three subjects belonging to the same family affected by basal cell nevus syndrome. They emphasise that the typical triad of the syndrome (basal-cell nevus, maxillary keratocysts and skeletal anomalies) was present in the subjects examined. The skeletal anomalies were very evident in the eldest daughter, negligible in the younger daughter and absent in the mother. Only two cysts closely related to erupting teeth were removed in the younger daughter between 1992 and 1999, whereas the basal-cell nevi were clearly prevalent compared to other pathological manifestations. Moreover, the authors observe that the cysts increased in volume much more rapidly in the daughters than in the mother: having identified the histological components of the cysts, this phenomenon might be linked to the age of the subjects examined.
Mazzola
G
G
Divisione di Odontostomatologia, Azienda Ospedaliera Villa Sofia, CTO, Palermo.
Galioto
V
V
Mazzola
U
U
D000293:Adolescent; D001478:Basal Cell Nevus Syndrome / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008441:Maxillary Neoplasms / Q000235:genetics*; D008875:Middle Aged