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Abstract
Cowden's syndrome or multiple hamartoma and neoplasia syndrome is a hereditary disorder
combining multiple hamartomas of many organs. These hamartomas are derived from ectodermal,
mesodermal, and endodermal tissues and may undergo spontaneous malignant changes.
Small papular cutaneous lesions and papillomatous outgrowth and papular lesions of
the oral mucosa are the most predominant features of the syndrome. Since the orocutaneous
lesions often precede the associated malignant degenerations, they may serve as a
diagnostic signal for the probably developing neoplasia in other organs. The presence
of extensive papillomatous and papular lesions in the oral mucosa combined with skin
lesions should therefore alert the clinician to suspect the presence of Cowden's syndrome,
the early diagnosis of which is important in order that the concomitant malignant
changes might be discovered at the earliest possible moment. Four cases of Cowden's
disease are presented, with emphasis on the oral manifestations of this syndrome.
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© 1985 Published by Elsevier Inc.
