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Olmsted Syndrome: Rare Occurrence in Four Siblings

Olmsted syndrome is a very rare and severe cicatrizing keratoderma associated with periorificial lesion. Most cases are sporadic but familial occurrence has been also seen. Till now around 73 cases have been reported and none of the reported cases have 4 siblings affected from this disease. We are r...

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Autores principales: Bukharia, Atishay, Komal, Sweta, Sudhanan, V Madhu, Chaudhary, Shyam Sundar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4885203/
https://www.ncbi.nlm.nih.gov/pubmed/27293270
http://dx.doi.org/10.4103/0019-5154.182467
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author Bukharia, Atishay
Komal, Sweta
Sudhanan, V Madhu
Chaudhary, Shyam Sundar
author_facet Bukharia, Atishay
Komal, Sweta
Sudhanan, V Madhu
Chaudhary, Shyam Sundar
author_sort Bukharia, Atishay
collection PubMed
description Olmsted syndrome is a very rare and severe cicatrizing keratoderma associated with periorificial lesion. Most cases are sporadic but familial occurrence has been also seen. Till now around 73 cases have been reported and none of the reported cases have 4 siblings affected from this disease. We are reporting cases of 4 siblings of age 30 year female, 26 year female, 20 year male and 10 year male who were born to a third degree consangueinous marriage and presented with palmoplantar keratoderma, periorificial hyperkeratosis, flexion deformity, pseudoainhum and contracture of digits. There was no cardiac involvement. Hence, the diagnosis of Olmsted syndrome was made and all four patients were non responsive to treatment which included topical corticosteroid, topical salicylic acid, systemic isotretinoin, systemic acitretin and oral zinc in child.
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spelling pubmed-48852032016-06-10 Olmsted Syndrome: Rare Occurrence in Four Siblings Bukharia, Atishay Komal, Sweta Sudhanan, V Madhu Chaudhary, Shyam Sundar Indian J Dermatol E-IJD Case Report Olmsted syndrome is a very rare and severe cicatrizing keratoderma associated with periorificial lesion. Most cases are sporadic but familial occurrence has been also seen. Till now around 73 cases have been reported and none of the reported cases have 4 siblings affected from this disease. We are reporting cases of 4 siblings of age 30 year female, 26 year female, 20 year male and 10 year male who were born to a third degree consangueinous marriage and presented with palmoplantar keratoderma, periorificial hyperkeratosis, flexion deformity, pseudoainhum and contracture of digits. There was no cardiac involvement. Hence, the diagnosis of Olmsted syndrome was made and all four patients were non responsive to treatment which included topical corticosteroid, topical salicylic acid, systemic isotretinoin, systemic acitretin and oral zinc in child. Medknow Publications & Media Pvt Ltd 2016 /pmc/articles/PMC4885203/ /pubmed/27293270 http://dx.doi.org/10.4103/0019-5154.182467 Text en Copyright: © 2016 Indian Journal of Dermatology http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
spellingShingle E-IJD Case Report
Bukharia, Atishay
Komal, Sweta
Sudhanan, V Madhu
Chaudhary, Shyam Sundar
Olmsted Syndrome: Rare Occurrence in Four Siblings
title Olmsted Syndrome: Rare Occurrence in Four Siblings
title_full Olmsted Syndrome: Rare Occurrence in Four Siblings
title_fullStr Olmsted Syndrome: Rare Occurrence in Four Siblings
title_full_unstemmed Olmsted Syndrome: Rare Occurrence in Four Siblings
title_short Olmsted Syndrome: Rare Occurrence in Four Siblings
title_sort olmsted syndrome: rare occurrence in four siblings
topic E-IJD Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4885203/
https://www.ncbi.nlm.nih.gov/pubmed/27293270
http://dx.doi.org/10.4103/0019-5154.182467
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AT sudhananvmadhu olmstedsyndromerareoccurrenceinfoursiblings
AT chaudharyshyamsundar olmstedsyndromerareoccurrenceinfoursiblings