Basal cell nevus syndrome: guidelines for early detection.
Basal cell nevus syndrome is an autosomal dominant condition with complete penetrance and variable expressivity. It is characterized by five major components, including multiple nevoid basal cell carcinomas, jaw cysts, congenital skeletal abnormalities, ectopic calcifications, and plantar or palmar pits. Other features include a host of benign tumors, ocular defects, and cleft lip and palate. Guidelines for diagnosis include a family history, careful oral and skin examinations, chest and skull radiographs, panoramic radiographs of the jaw, magnetic resonance imaging of the brain, and pelvic ultrasonography in women.
Bitar
George J
GJ
Department of Plastic Surgery, Albert Einstein College of Medicine, Bronx, New York, USA. GBITAR007@aol.com
Herman
Charles K
CK
Dahman
Mohammed I
MI
Hoard
Martin A
MA
D000293:Adolescent; D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D005260:Female; D006801:Humans; D010819:Physician's Role; D012008:Recurrence; D012867:Skin / Q000473:pathology