Basal cell nevus syndrome

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.

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.

Symptoms

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.

Treatment

Very frequent examinations by a dermatologist are critical, so that skin cancers may be treated while they are still small.

Expectations (prognosis)

Frequent follow-up with a variety of doctors is vital to achieving a good outcome.

Review

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

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

ADAM Medical Encyclopedia

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