D-Penicillamine Induced Degenerative Dermopathy in a Patient with Wilson Disease

• Main Authors: Rui Pedro Santos, Joana Gomes, Celeste Brito
• Format: Article
• Language: English
• Published: Sociedade Portuguesa de Dermatologia e Venereologia 2018-05-01
• Series: Revista da Sociedade Portuguesa de Dermatologia e Venereologia
• Subjects: Penicillamine/adverse effects; Hepatolenticular Degeneration; Pseudoxanthoma Elasticum; Skin Diseases/chemically induced
• Online Access: https://revista.spdv.com.pt/index.php/spdv/article/view/826
• ISSN: 2182-2395; 2182-2409

D-penicillamine induced degenerative dermatosis include, among others, elastosis perforans serpiginosa, and pseudo- -pseudoxanthoma elasticum. Elastosis perforans serpiginosa is a rare perforating disease characterized by transepidermal elimination of abnormal elastic fibers. This condition can be idiopathic, reactive or induced by D-penicillamine, commonly used for the treatment of Wilson disease, cystinuria, rheumatoid arthritis or systemic sclerosis. Cutaneous manifestations resembling pseudoxanthoma elasticum but lacking familiar history and ABCC6 mutations have been identified as a D-penicillamine induced dermopathy and called pseudo-pseudoxanthoma elasticum. The authors present a 17-year-old caucasian female treated for several years with D-penicillamine for Wilson disease who developed asymptomatic papules, some hyperkeratotic skin-colored and other soft and yellowish, on the cervical region and face. Histopathology showed transepidermal elimination of numerous, branched, sawtooth-like elastic fibers. These findings suggested a D-penicillamine induced dermopathy and the authors considered the diagnosis of both elastosis perforans serpiginosa and pseudo- -pseudoxanthoma elasticum in the same patient. The drug was switched to zinc acetate. No newer lesions appeared thereafter but previous lesions persisted at the 1 year follow-up.