Basal cell nevus syndrome - Symptom
Nevoid basal cell carcinoma syndrome; Gorlin syndrome
The hallmark of this disorder is the appearance of a type of skin cancer known as basal cell carcinoma at or around puberty.
- Broad nose
- Cleft palate
- Heavy, protruding brow
- Jaw that sticks out (in some cases)
- Wide-set eyes
The condition may affect the nervous system and lead to:
The condition also leads to bone defects, including:
Signs and tests:
There may be a family history of basal cell nevus syndrome and a past history of basal cell skin cancers.
Tests may reveal:
- Brain tumors
- Cysts in the jaw, which can lead to abnormal tooth development or jaw fractures
- Defects in the colored part (iris) or lens of the eye
- Head swelling due to fluid on the brain (hydrocephalus)
- Rib abnormalities
Tests that may be done include:
- 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.
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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