Open AccessPompholyx-like eruptions induced by penicillamine in a patient with wilson's disease
Author(s) -
Diana Elizabeth Devassy,
Sujata Raj Harshad,
Harshad Devarbhai
Publication year - 2019
Publication title -
indian journal of dermatology/indian journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.395
H-Index - 36
eISSN - 1998-3611
pISSN - 0019-5154
DOI - 10.4103/ijd.ijd_328_18
Subject(s) - penicillamine , medicine , dermatology , adverse effect , blisters , pseudoxanthoma elasticum , histopathology , copper metabolism , wilson's disease , disease , copper , pathology , immunology , chemistry , organic chemistry
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.