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Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations

Aim. To present a case of Cowden's syndrome and emphasize the importance of continued cancer surveillance in these patients. Cowden syndrome is an inherited autosomal dominant trait with incomplete penetrance and a range of expressivity. It is characterized by multiple hamartomas and neoplasms....

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Autores principales: Chippagiri, Prashanthi, Banavar Ravi, Spoorthi, Patwa, Neha
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3787619/
https://www.ncbi.nlm.nih.gov/pubmed/24151559
http://dx.doi.org/10.1155/2013/315109
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author Chippagiri, Prashanthi
Banavar Ravi, Spoorthi
Patwa, Neha
author_facet Chippagiri, Prashanthi
Banavar Ravi, Spoorthi
Patwa, Neha
author_sort Chippagiri, Prashanthi
collection PubMed
description Aim. To present a case of Cowden's syndrome and emphasize the importance of continued cancer surveillance in these patients. Cowden syndrome is an inherited autosomal dominant trait with incomplete penetrance and a range of expressivity. It is characterized by multiple hamartomas and neoplasms. Mucocutaneous features include trichilemmomas, oral mucosal papillomatosis, acral keratosis, and palmoplantar keratosis. Here, we report a case of Cowdens syndrome of a 30-year-old female patient who came with a complaint of multiple growths in the oral cavity of a three-month duration. On examination, multiple skin-colored, flat-topped papules over her forehead and right malar bone and multiple papillomatous papules involving all the mucosal surfaces intraorally were observed. This syndrome is associated with the development of several types of malignancies, especially breast carcinoma and thyroid carcinoma, which is why early recognition and regular and vigilant surveillance of individuals with the syndrome are important.
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spelling pubmed-37876192013-10-22 Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations Chippagiri, Prashanthi Banavar Ravi, Spoorthi Patwa, Neha Case Rep Dent Case Report Aim. To present a case of Cowden's syndrome and emphasize the importance of continued cancer surveillance in these patients. Cowden syndrome is an inherited autosomal dominant trait with incomplete penetrance and a range of expressivity. It is characterized by multiple hamartomas and neoplasms. Mucocutaneous features include trichilemmomas, oral mucosal papillomatosis, acral keratosis, and palmoplantar keratosis. Here, we report a case of Cowdens syndrome of a 30-year-old female patient who came with a complaint of multiple growths in the oral cavity of a three-month duration. On examination, multiple skin-colored, flat-topped papules over her forehead and right malar bone and multiple papillomatous papules involving all the mucosal surfaces intraorally were observed. This syndrome is associated with the development of several types of malignancies, especially breast carcinoma and thyroid carcinoma, which is why early recognition and regular and vigilant surveillance of individuals with the syndrome are important. Hindawi Publishing Corporation 2013 2013-09-16 /pmc/articles/PMC3787619/ /pubmed/24151559 http://dx.doi.org/10.1155/2013/315109 Text en Copyright © 2013 Prashanthi Chippagiri et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Chippagiri, Prashanthi
Banavar Ravi, Spoorthi
Patwa, Neha
Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations
title Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations
title_full Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations
title_fullStr Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations
title_full_unstemmed Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations
title_short Multiple Hamartoma Syndrome with Characteristic Oral and Cutaneous Manifestations
title_sort multiple hamartoma syndrome with characteristic oral and cutaneous manifestations
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3787619/
https://www.ncbi.nlm.nih.gov/pubmed/24151559
http://dx.doi.org/10.1155/2013/315109
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