Elastosis Perforans Serpiginosa and Wilson Disease: A Rare but Predictable Consequence of Long-term Therapy with D-Penicillamine

Authors

  • André Castro Pinho Serviço de Dermatologia. Hospitais da Universidade de Coimbra. Centro Hospitalar e Universitário de Coimbra. Coimbra.
  • José Carlos Cardoso Serviço de Dermatologia. Hospitais da Universidade de Coimbra. Centro Hospitalar e Universitário de Coimbra. Coimbra.
  • Miguel Gouveia Serviço de Dermatologia. Hospitais da Universidade de Coimbra. Centro Hospitalar e Universitário de Coimbra. Coimbra.
  • Hugo Oliveira Serviço de Dermatologia. Hospitais da Universidade de Coimbra. Centro Hospitalar e Universitário de Coimbra. Coimbra.

DOI:

https://doi.org/10.20344/amp.6749

Keywords:

Chelating Agents/therapeutic use, Hepatolenticular Degeneration, Penicillamine, Skin Diseases/chemically induced.

Abstract

Elastosis perfurans serpiginosa is a rare perforating dermatosis found primarily in adolescents and young adults, characterized by transepidermal elimination of abnormal elastic fibers. The only drug known capable of inducing elastosis perfurans serpiginosa is D-penicillamine. We report the case of a 52 year-old woman with keratotic papules arranged in an annular pattern with central clearing and centrifugal growth, located in the anterior cervical region. The patient was chronically treated with D-penicillamine for Wilson disease. Lesion biopsy showed transepidermal elimination of thickened, eosinophilic, branched, sawtooth-like elastic fibers. The clinical and pathological findings were consistent with elastosis perfurans serpiginosa secondary to D-penicillamine. It is estimated that elastosis perfurans serpiginosa occurs in 1% of patients treated with D-penicillamine. By blocking directly or indirectly the desmosine cross-links between elastin molecules, D-penicillamine leads to the synthesis of abnormal dermal and extracutaneous elastic fibers. Elastosis perfurans serpiginosa may be the first manifestation of a multisystemic degenerative process of elastic connective tissue.

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Published

2016-03-31

How to Cite

1.
Pinho AC, Cardoso JC, Gouveia M, Oliveira H. Elastosis Perforans Serpiginosa and Wilson Disease: A Rare but Predictable Consequence of Long-term Therapy with D-Penicillamine. Acta Med Port [Internet]. 2016 Mar. 31 [cited 2024 Mar. 28];29(3):227-30. Available from: https://actamedicaportuguesa.com/revista/index.php/amp/article/view/6749

Issue

Section

Case Report