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Basal cell nevus syndrome

• Overview
• Symptoms
• Treatment
• Prevention

Alternative Names:

Nevoid basal cell carcinoma syndrome; Gorlin's syndrome

Symptoms:

• Family history of basal cell nevus syndrome
• Past diagnosis of multiple basal cell skin cancers
• Characteristic facial appearance including wide-set eyes, broad nasal bridge, heavy ridge over eyes, and protruding jaw
• Defects in iris of the eye
• Opaque lens of eye ( cataracts )
• Blindness
• Abnormal teeth with pits in the enamel
• Skeletal abnormalities

Signs and tests:

There is often a family history of basal cell nevus syndrome.

X-rays may reveal bone cysts in the jaw, mineral deposits in the brain, and multiple bone abnormalities.

Skin biopsy of tumors may be used to determine if they are basal cell carcinoma.

Genetic testing may demonstrate an abnormality in some patients with this disorder.

• Review Date: 7/27/2006
• Reviewed By: Shlomit Halachmi, MD, PhD, Private Practice Specializing in Dermatology, Somerville, MA. Review provided by VeriMed Healthcare Network.

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