GARDNER SYNDROME

By Gary M. White, MD

The Gardner syndrome (GS) is a symptom complex characterized by intestinal polyposis, mandibular, maxillary and long bone osteomas, fibromas, lipomas, dental abnormalities (mandibular cysts, impacted teeth, and supernumerary teeth) and epidermal inclusion cysts.

Etiology          

It is thought that Gardner syndrome is a variant of familial adenomatous polyposis. The genetic defect of both diseases is the APC (adenomatous polyposis coli) gene found on chromosome 5 which is a classic tumor-suppressor gene.

Treatment

Given that the intestinal polyps have nearly 100% malignant potential if untreated, the important management issues are handled by gastroenterology. Cutaneous cysts may be surgically excised.

References

Journal of the American Academy of Dermatology Volume 68, Issue 2, Pages 189.e1–189.e21, February 2013

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