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Otological lesions in pachyonychia congenita syndrome

Published online by Cambridge University Press:  29 June 2007

Ricardo Ferreira Bento ,
Maria Heleba Guatimosim ,
Rogério de Leão Bensadon ,
Tanit Ganz Sanchez  and
Richard Louis Voegels
Ricardo Ferreira Bento*
Affiliation:
Department of Otolaryngology, University of São Paulo, Sãa Paulo, Brazil.
Maria Heleba Guatimosim
Affiliation:
Department of Otolaryngology, University of São Paulo, Sãa Paulo, Brazil.
Rogério de Leão Bensadon
Affiliation:
Department of Otolaryngology, University of São Paulo, Sãa Paulo, Brazil.
Tanit Ganz Sanchez
Affiliation:
Department of Otolaryngology, University of São Paulo, Sãa Paulo, Brazil.
Richard Louis Voegels
Affiliation:
Department of Otolaryngology, University of São Paulo, Sãa Paulo, Brazil.
*
Address for correspondence: Ricardo Ferreira Bento, M.D., Ph.D., Rua Dr. Enéas de Carvalho Aguiar 225 – 6° andar – sala 6002, São Paulo- Brazil – 05403-000. Fax: (5511) 280-0299
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Abstract

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The authores report a case of patient with pachyonychia syndrome, a rare genedermatosis inherited as an autosomal dominant trait, who also had otological lesions beyond the other classic signs and symptoms of the syndrome. Many kinds of treatment have already been proposed, but all failed to show satisfactory results. A new, cheap and easy-to-use treatment was developed in this study, using keratoplastics interpolated with humectant lotion for 90 days. The results after three years of follow-up are still thoroughly satisfactory.

Keywords

Ear, externalKeratosis
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 110 , Issue 12 , December 1996 , pp. 1145 - 1147
Copyright
Copyright © JLO (1984) Limited 1996

Footnotes

Presented during the Annual Meeting of American Academy of Otolaryngology – Head and neck Surgery, in Sab Diego, California, 1994.

References

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Kumer, L., Loos, H. O. (1935) Congenital pachyonychia (Riehl type). Wiener Wochenschrift 48: 174–178.Google Scholar
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