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.
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 basal cell carcinoma at or around puberty.
The condition may affect the nervous system and lead to:
The condition also leads to bone defects, including:
The person may have a family history of basal cell nevus syndrome and several basal cell skin cancers in the past.
Tests may reveal:
Tests that may be done include:
Very frequent examinations by a dermatologist are critical, so that skin cancers may be treated while they are still small.
Persons with this condition may also be seen and treated by several other specialists, depending on what part of the body is affected. For example, a cancer specialist (oncologist) may treat internal tumors, and an orthopedic surgeon may help treat bone problems.
Frequent follow-up with a variety of doctors is vital to achieving a good outcome.
Call for an appointment with your health care provider if:
Couples with a family history of this syndrome might consider genetic counseling before becoming pregnant.
Avoiding the sun and using sunscreen are necessary to help prevent new basal cell skin cancers.
Avoid ionizing radiation such as x-rays. People with this condition are very sensitive to radiation, and exposure can lead to skin cancers.
Nevoid basal cell carcinoma syndrome; Gorlin syndrome