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Focal palmoplantar and gingival keratosis – A rare genodermatoses: Case report

Focal palmoplantar and gingival keratosis syndrome is a rare dominant inherited disease with an early onset in life. Clinically, the condition is characterized by pressure related thickening of the epidermis of the palms and soles, usually accompanied by pain and different levels of skin involvement...

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Autores principales: Gerber-Mora, Roberto, Jajam-Maturana, Martín, Fernández-Moraga, Alejandra, Martinez-Flores, Rene
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medicina Oral S.L. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7680573/
https://www.ncbi.nlm.nih.gov/pubmed/33262878
http://dx.doi.org/10.4317/jced.57528
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author Gerber-Mora, Roberto
Jajam-Maturana, Martín
Fernández-Moraga, Alejandra
Martinez-Flores, Rene
author_facet Gerber-Mora, Roberto
Jajam-Maturana, Martín
Fernández-Moraga, Alejandra
Martinez-Flores, Rene
author_sort Gerber-Mora, Roberto
collection PubMed
description Focal palmoplantar and gingival keratosis syndrome is a rare dominant inherited disease with an early onset in life. Clinically, the condition is characterized by pressure related thickening of the epidermis of the palms and soles, usually accompanied by pain and different levels of skin involvement and thickness between patients. Recently, we observed a 38-year-old woman with multiple non-removable, painless white plaques of variable size and thickness on the attached gingiva and a white plaque widespread across the hard palate. By further questioning, the patient comments that she has thick yellowish focal plaques in both soles of her feet. Histopathological analysis revealed a hyperplastic and hyperorthokeratinized stratified squamous epithelium with basal hyperplasia. The spinous, granular and stratum corneum showed dispersed basophilic keratohyalin granules. At higher magnification, the keratinocytes contained paranuclear bodies, seen as round eosinophilic condensation that indented the nuclei. Based on these findings the final diagnosis was rendered as focal palmoplantar and gingival keratosis. Key words:Genodermatoses, white plaque, dyskeratosis, gingiva.
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spelling pubmed-76805732020-11-30 Focal palmoplantar and gingival keratosis – A rare genodermatoses: Case report Gerber-Mora, Roberto Jajam-Maturana, Martín Fernández-Moraga, Alejandra Martinez-Flores, Rene J Clin Exp Dent Case Report Focal palmoplantar and gingival keratosis syndrome is a rare dominant inherited disease with an early onset in life. Clinically, the condition is characterized by pressure related thickening of the epidermis of the palms and soles, usually accompanied by pain and different levels of skin involvement and thickness between patients. Recently, we observed a 38-year-old woman with multiple non-removable, painless white plaques of variable size and thickness on the attached gingiva and a white plaque widespread across the hard palate. By further questioning, the patient comments that she has thick yellowish focal plaques in both soles of her feet. Histopathological analysis revealed a hyperplastic and hyperorthokeratinized stratified squamous epithelium with basal hyperplasia. The spinous, granular and stratum corneum showed dispersed basophilic keratohyalin granules. At higher magnification, the keratinocytes contained paranuclear bodies, seen as round eosinophilic condensation that indented the nuclei. Based on these findings the final diagnosis was rendered as focal palmoplantar and gingival keratosis. Key words:Genodermatoses, white plaque, dyskeratosis, gingiva. Medicina Oral S.L. 2020-11-01 /pmc/articles/PMC7680573/ /pubmed/33262878 http://dx.doi.org/10.4317/jced.57528 Text en Copyright: © 2020 Medicina Oral S.L. http://creativecommons.org/licenses/by/2.5/ This is an open-access article distributed under the terms of 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
Gerber-Mora, Roberto
Jajam-Maturana, Martín
Fernández-Moraga, Alejandra
Martinez-Flores, Rene
Focal palmoplantar and gingival keratosis – A rare genodermatoses: Case report
title Focal palmoplantar and gingival keratosis – A rare genodermatoses: Case report
title_full Focal palmoplantar and gingival keratosis – A rare genodermatoses: Case report
title_fullStr Focal palmoplantar and gingival keratosis – A rare genodermatoses: Case report
title_full_unstemmed Focal palmoplantar and gingival keratosis – A rare genodermatoses: Case report
title_short Focal palmoplantar and gingival keratosis – A rare genodermatoses: Case report
title_sort focal palmoplantar and gingival keratosis – a rare genodermatoses: case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7680573/
https://www.ncbi.nlm.nih.gov/pubmed/33262878
http://dx.doi.org/10.4317/jced.57528
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