Abstract
Clinical signs and features include:
Variant of familial adenomatous polyposis
Clinically presents with polyps in the gastrointestinal tract (GI), skull/mandible osteomas, dental abnormalities (multiple impacted or unerupted teeth), fibromas, desmoid tumors, and congenital hypertrophy of the retinal epithelium bilaterally
First sign is often epidermoid cysts, which can present about 10 years before the polyps
Cysts appear just before puberty on the face, extremities, and scalp
Cysts are usually asymptomatic but can present with pruritus, inflammation, and rupture
Other skin manifestations include lipomas and leiomyomas
Colonic polyps are associated with increased risk of colon cancer
Pathogenesis of this disease involves a genetic mutation:
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References
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Zakko, L., Finch, J., Rothe, M.J., Grant-Kels, J.M. (2013). Gardner Syndrome: Dermatological Features. In: Wu, G., Selsky, N., Grant-Kels, J. (eds) Atlas of Dermatological Manifestations of Gastrointestinal Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6191-3_42
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DOI: https://doi.org/10.1007/978-1-4614-6191-3_42
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