Basal cell nevus syndrome : Overview, Causes, & Risk Factors

Alternate Names : Nevoid basal cell carcinoma syndrome, Gorlin syndrome

Definition

Basal cell nevus syndrome is a group of defects, passed down through families, that involve the skin, nervous system, eyes, endocrine glands, and bones.

The condition causes an unusual facial appearance and a higher risk of skin cancers.

Overview, Causes, & Risk Factors

Basal cell nevus syndrome is a rare genetic condition. The gene linked to the syndrome is passed down through families as an autosomal dominant trait. That means you will get the syndrome if either parent passes the gene down to you.

Pictures & Images

Basal cell nevus syndrome – close-up of palm

Basal cell nevus syndrome is an inherited disorder characterized by wide-set eyes, saddle nose, frontal bossing (prominent forehead), prognathism (prominent chin), numerous basal cell carcinomas, and skeletal abnormalities. Skin manifestations include pits in the palms and soles, and numerous basal cell carcinomas. This picture is a close-up of the pits found in the palm of an individual with basal cell nevus syndrome.

Basal cell nevus syndrome – plantar pits

Basal cell nevus syndrome - plantar pits

Basal cell nevus syndrome is an inherited disorder characterized by wide-set eyes, saddle nose, frontal bossing (prominent forehead), prognathism (prominent chin), and skeletal abnormalities. Skin manifestations include pits in the palms and soles, and numerous basal cell carcinomas (skin cancers). This picture is a close-up of the pits found on the sole of the foot of an individual with basal cell nevus syndrome.

Basal cell nevus syndrome – face and hand

Basal cell nevus syndrome - face and hand

Basal cell nevus syndrome is an inherited disorder characterized by wide-set eyes, saddle nose, frontal bossing (prominent forehead), prognathism (prominent chin), numerous basal cell carcinomas (a type of skin cancer), and skeletal abnormalities. This individual has multiple flesh-colored, dome-shaped bumps on the face which are basal cell cancers, and palmar pits.

Basal cell nevus syndrome

Basal cell nevus syndrome

Basal cell nevus syndrome is an inherited disorder which inclines the patient to development of multiple basal cell carcinomas, most prevalent for unknown reasons around the eyes and nose. Here they appear as slightly larger than pinpoint papules on the eye lid.

Basal cell nevus syndrome – face

Basal cell nevus syndrome - face

The basal cell nevus syndrome has associated with it odontogenic cysts of the jaws, pitted depressions of the hands and feet (tiny basal cells), and osseous anomalies of the skeleton. Care is given by removal of the carcinomata on a regular basis and genetic counseling, since this is an autosomal dominatly inherited disorder.


Review Date : 8/2/2009
Reviewed By : Michael Lehrer, MD, Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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