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
Descripción
Sumario: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.