Nevoid basal cell carcinoma syndrome; Gorlin syndrome
Definition of Basal cell nevus syndrome
Basal cell nevus syndrome is a group of defects, passed down through families, that involve the skin, nervous system, eyes, endocrine glands, and bones.
Causes, incidence, and 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.
The hallmark of this disorder is the appearance of a type of skin cancer known as at or around puberty.
Signs and tests
The person may have a family history of basal cell nevus syndrome and several basal cell skin cancers in the past.
Very frequent examinations by a dermatologist are critical, so that skin cancers may be treated while they are still small.
Frequent follow-up with a variety of doctors is vital to achieving a good outcome.
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. – 8/2/2009