Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Devassy, Diana Elizabeth, Harshad, Sujata Raj, Devarbhai, Harshad
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2019
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
_version_ 1783447012064100352
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
work_keys_str_mv AT devassydianaelizabeth pompholyxlikeeruptionsinducedbypenicillamineinapatientwithwilsonsdisease
AT harshadsujataraj pompholyxlikeeruptionsinducedbypenicillamineinapatientwithwilsonsdisease
AT devarbhaiharshad pompholyxlikeeruptionsinducedbypenicillamineinapatientwithwilsonsdisease