Devassy Diana Elizabeth, Harshad Sujata Raj, Devarbhai Harshad
Department of Dermatology, St. Johns Medical College Hospital, Bengaluru, Karnataka, India.
Department of Gastroenterology, St. Johns Medical College Hospital, Bengaluru, Karnataka, India.
Indian J Dermatol. 2019 Jul-Aug;64(4):321-323. doi: 10.4103/ijd.IJD_328_18.
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.
威尔逊病是一种遗传性铜代谢紊乱疾病，会导致铜在各个器官中过度蓄积，肝脏是主要受累部位。D-青霉胺（DPA）作为一种螯合剂是治疗的主要药物；然而，长期使用会引起众多不良反应。与DPA相关的主要不良反应包括胶原合成障碍，如弹性假黄瘤样病变、自身免疫性水疱病和荨麻疹样病变。在此，我们报告一名年轻女孩，在开始使用DPA几个月内出现了广泛的汗疱疹样水疱，组织病理学显示为海绵状反应。停用DPA并开始口服类固醇后，病变有所改善。
