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Pompholyx-Like Eruptions Induced by Penicillamine in a Patient with Wilson's Disease
Wilson's disease is an inherited disorder of copper metabolism that results in excessive accumulation of copper in various organs, with liver being the primary site of involvement. D-penicillamine (DPA) as a chelating agent forms the mainstay of therapy; however, it can cause a myriad of advers...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer - Medknow
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6714198/ https://www.ncbi.nlm.nih.gov/pubmed/31516144 http://dx.doi.org/10.4103/ijd.IJD_328_18 |
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author | Devassy, Diana Elizabeth Harshad, Sujata Raj Devarbhai, Harshad |
author_facet | Devassy, Diana Elizabeth Harshad, Sujata Raj Devarbhai, Harshad |
author_sort | Devassy, Diana Elizabeth |
collection | PubMed |
description | Wilson's disease is an inherited disorder of copper metabolism that results in excessive accumulation of copper in various organs, with liver being the primary site of involvement. D-penicillamine (DPA) as a chelating agent forms the mainstay of therapy; however, it can cause a myriad of adverse effects on long-term use. The major adverse effects reported with DPA include disorders of collagen synthesis, such as pseudoxanthoma elasticum-like lesions, autoimmune blistering diseases, and urticarial lesions. Here, we report a young girl who developed extensive pompholyx-like blisters within a few months of starting DPA which on histopathology showed a spongiotic reaction. The lesions improved on stopping DPA and initiation of oral steroids. |
format | Online Article Text |
id | pubmed-6714198 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Wolters Kluwer - Medknow |
record_format | MEDLINE/PubMed |
spelling | pubmed-67141982019-09-12 Pompholyx-Like Eruptions Induced by Penicillamine in a Patient with Wilson's Disease Devassy, Diana Elizabeth Harshad, Sujata Raj Devarbhai, Harshad Indian J Dermatol Case Report Wilson's disease is an inherited disorder of copper metabolism that results in excessive accumulation of copper in various organs, with liver being the primary site of involvement. D-penicillamine (DPA) as a chelating agent forms the mainstay of therapy; however, it can cause a myriad of adverse effects on long-term use. The major adverse effects reported with DPA include disorders of collagen synthesis, such as pseudoxanthoma elasticum-like lesions, autoimmune blistering diseases, and urticarial lesions. Here, we report a young girl who developed extensive pompholyx-like blisters within a few months of starting DPA which on histopathology showed a spongiotic reaction. The lesions improved on stopping DPA and initiation of oral steroids. Wolters Kluwer - Medknow 2019 /pmc/articles/PMC6714198/ /pubmed/31516144 http://dx.doi.org/10.4103/ijd.IJD_328_18 Text en Copyright: © 2019 Indian Journal of Dermatology http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. |
spellingShingle | Case Report Devassy, Diana Elizabeth Harshad, Sujata Raj Devarbhai, Harshad Pompholyx-Like Eruptions Induced by Penicillamine in a Patient with Wilson's Disease |
title | Pompholyx-Like Eruptions Induced by Penicillamine in a Patient with Wilson's Disease |
title_full | Pompholyx-Like Eruptions Induced by Penicillamine in a Patient with Wilson's Disease |
title_fullStr | Pompholyx-Like Eruptions Induced by Penicillamine in a Patient with Wilson's Disease |
title_full_unstemmed | Pompholyx-Like Eruptions Induced by Penicillamine in a Patient with Wilson's Disease |
title_short | Pompholyx-Like Eruptions Induced by Penicillamine in a Patient with Wilson's Disease |
title_sort | pompholyx-like eruptions induced by penicillamine in a patient with wilson's disease |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6714198/ https://www.ncbi.nlm.nih.gov/pubmed/31516144 http://dx.doi.org/10.4103/ijd.IJD_328_18 |
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