Cargando…

Pachyonychia congenita with late onset (PC tarda)

Pachyonychia congenita is a rare type of ectodermal dysplasia further classified into 4 types. Cutaneous manifestations seen in most of the cases of Pachyonychia congenita include palmoplantar keratoderma, follicular hyperkeratosis, wedge shaped nails, oral leukokeratosis and woolly hair. A 25-year-...

Descripción completa

Detalles Bibliográficos
Autores principales: Sravanthi, A., Srivalli, P., Gopal, K. V. T., Rao, T. Narayana
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/PMC4976406/
https://www.ncbi.nlm.nih.gov/pubmed/27559502
http://dx.doi.org/10.4103/2229-5178.185463
_version_ 1782446870478454784
author Sravanthi, A.
Srivalli, P.
Gopal, K. V. T.
Rao, T. Narayana
author_facet Sravanthi, A.
Srivalli, P.
Gopal, K. V. T.
Rao, T. Narayana
author_sort Sravanthi, A.
collection PubMed
description Pachyonychia congenita is a rare type of ectodermal dysplasia further classified into 4 types. Cutaneous manifestations seen in most of the cases of Pachyonychia congenita include palmoplantar keratoderma, follicular hyperkeratosis, wedge shaped nails, oral leukokeratosis and woolly hair. A 25-year-old male presented to us with thickened nails and scanty scalp hair. On examination, we noticed hyperkeratotic plaques over both the soles, palmoplantar hyperhidrosis and yellowish discoloration, wedging with subungual hyperkeratosis of all the nails. Follicular hyperkeratotic papules and steatocystoma multiplex were also observed over the scalp and face. The patient had history of natal teeth and on dental examination, lower central incisors were absent. All cutaneous changes in our case had manifested first in the 2(nd) decade except for natal teeth. All the above features suggested the diagnosis of pachyonychia congenita with late onset (PC tarda), which is an infrequently reported rare variant.
format Online
Article
Text
id pubmed-4976406
institution National Center for Biotechnology Information
language English
publishDate 2016
publisher Medknow Publications & Media Pvt Ltd
record_format MEDLINE/PubMed
spelling pubmed-49764062016-08-24 Pachyonychia congenita with late onset (PC tarda) Sravanthi, A. Srivalli, P. Gopal, K. V. T. Rao, T. Narayana Indian Dermatol Online J Case Report Pachyonychia congenita is a rare type of ectodermal dysplasia further classified into 4 types. Cutaneous manifestations seen in most of the cases of Pachyonychia congenita include palmoplantar keratoderma, follicular hyperkeratosis, wedge shaped nails, oral leukokeratosis and woolly hair. A 25-year-old male presented to us with thickened nails and scanty scalp hair. On examination, we noticed hyperkeratotic plaques over both the soles, palmoplantar hyperhidrosis and yellowish discoloration, wedging with subungual hyperkeratosis of all the nails. Follicular hyperkeratotic papules and steatocystoma multiplex were also observed over the scalp and face. The patient had history of natal teeth and on dental examination, lower central incisors were absent. All cutaneous changes in our case had manifested first in the 2(nd) decade except for natal teeth. All the above features suggested the diagnosis of pachyonychia congenita with late onset (PC tarda), which is an infrequently reported rare variant. Medknow Publications & Media Pvt Ltd 2016 /pmc/articles/PMC4976406/ /pubmed/27559502 http://dx.doi.org/10.4103/2229-5178.185463 Text en Copyright: © Indian Dermatology Online Journal 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 Case Report
Sravanthi, A.
Srivalli, P.
Gopal, K. V. T.
Rao, T. Narayana
Pachyonychia congenita with late onset (PC tarda)
title Pachyonychia congenita with late onset (PC tarda)
title_full Pachyonychia congenita with late onset (PC tarda)
title_fullStr Pachyonychia congenita with late onset (PC tarda)
title_full_unstemmed Pachyonychia congenita with late onset (PC tarda)
title_short Pachyonychia congenita with late onset (PC tarda)
title_sort pachyonychia congenita with late onset (pc tarda)
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4976406/
https://www.ncbi.nlm.nih.gov/pubmed/27559502
http://dx.doi.org/10.4103/2229-5178.185463
work_keys_str_mv AT sravanthia pachyonychiacongenitawithlateonsetpctarda
AT srivallip pachyonychiacongenitawithlateonsetpctarda
AT gopalkvt pachyonychiacongenitawithlateonsetpctarda
AT raotnarayana pachyonychiacongenitawithlateonsetpctarda