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Basal cell nevus syndrome - Overview

Alternative Names

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.

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

Causes, incidence, and risk factors:

Basal cell nevus syndrome is a rare genetic condition. The gene linked to the syndrome is known as PTCH ("patched").

The gene 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.

Basal cell nevus syndrome - close-up of palm

Basal cell nevus syndrome - plantar pits

Basal cell nevus syndrome - face and hand

Basal cell nevus syndrome

Basal cell nevus syndrome - face

Reviewed last on: 8/3/2011
Kevin Berman, MD, PhD, Associate, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Morelli JG. Tumors of the skin. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier;2011:chap 662.

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