1995 Volume 44 Issue 4 Pages 688-692
Basal cell nevus syndrome (BCNS) is a complex disease characterized by multiple cysts of the jaws, multiple basal cell tumors of the skin, a high incidence of skeletal abnormalities, and neurological disorders. In this paper, 2 cases of BCNS are reported and the results of clinical investigation of them are discussed.
The first case was a 16-year-old boy who visited our clinic in 1985 with the chief complaint of swelling in the molar region of the left mandible. Clinically, multiple cysts of the jaws, a broad nasal root, malocclusion, mild mandibular prognathism, and palmar pits were observed. Jaw cysts were enucleated. In 1988, occurrences of multiple basal cell nevi of the skin were confirmed. Furthermore, in 1993, recurrence of odontogenic keratocysts in the right mandible and maxilla were confirmed.
The other case was a 6-year-old girl who was referred to our clinic in 1991 because of swelling of the right face with infection. Radiographic examination showed multiple jaw cysts. In addition, general examination revealed frontal bossing, a broad nasal root, ocular hypertelorism, and plantar pits. She had a positive family history. Jaw cysts were enucleated, and she had no recurrence. However, odontogenic keratocysts in the left maxilla and in the left mandible were confirmed about 1 year after surgery on the right jaw cysts ; and in 1993, glioma of the brain was confirmed.
