{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T15:16:42Z","timestamp":1787239002307,"version":"build-2736575974"},"reference-count":19,"publisher":"S. Karger AG","issue":"1","license":[{"start":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T00:00:00Z","timestamp":1104537600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T00:00:00Z","timestamp":1104537600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dermatology"],"published-print":{"date-parts":[[2005]]},"abstract":"<i>Background:<\/i> Elastosis perforans serpiginosa (EPS) is a reactive perforating dermatosis characterized by the elimination of abnormal elastic fibers from the upper dermis through the epidermis. In a few cases, it occurs as a side effect of treatment by <i>D<\/i>-penicillamine (DPA). The first case of EPS induced by DPA was described in 1972 in a patient treated for Wilson\u2019s disease. Subsequently, cutaneous changes resembling pseudoxanthoma elasticum (PXE) were observed in patients treated with DPA and were reported as pseudo-PXE. <i>Case Report:<\/i> We report herein the clinical, pathological and ultrastructural study of 2 new cases of DPA-induced EPS and pseudo-PXE. These patients had been treated for Wilson\u2019s disease since 14 and 16 years, respectively. Characteristic abnormal elastic fibers were found on histopathological examination of both EPS and pseudo-PXE skin and confirmed by an ultrastructural study. There was no ABCC6 mutation. <i>Discussion:<\/i> Penicillamine is able to induce widespread, cutaneous and systemic, elastic fiber damage. Our patients present typical features of DPA-induced elastosis, presenting as EPS and pseudo-PXE. ABCC6 mutation is associated with PXE and, as expected, it was absent in our cases of pseudo-PXE. This elastopathy has been related to morphologic changes in elastic fibers secondary to prolonged therapy in most cases. DPA may interfere with elastin cross-linking through inhibition of the enzyme lysyl oxidase, or by formation of complexes with the cross-linked precursors, impairing a normal maturation of elastic fibers. However, no fatal complication of DPA-induced elastopathy has been reported so far. An improvement of the cutaneous lesions is expected after the drug discontinuation.<\/jats:p>","DOI":"10.1159\/000081487","type":"journal-article","created":{"date-parts":[[2004,12,14]],"date-time":"2004-12-14T08:00:22Z","timestamp":1103011222000},"page":"60-63","source":"Crossref","is-referenced-by-count":45,"title":["Elastosis perforans serpiginosa Associated with Pseudo-Pseudoxanthoma elasticum during Treatment of Wilson\u2019s Disease with Penicillamine"],"prefix":"10.1159","volume":"210","author":[{"given":"C\u00e9cile","family":"B\u00e9cuwe","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phane","family":"Dalle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Ronger-Savl\u00e9","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7ois","family":"Skowron","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitte","family":"Balme","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean","family":"Kanitakis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luc","family":"Thomas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2005,1,28]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1001%2Farchderm.113.5.630"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1046%2Fj.1365-2230.2001.00882.x"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1365-2230.1985.tb00588.x"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1365-2230.1988.tb00693.x"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1016%2F0002-9343%2856%2990066-3"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1016%2FS0190-9622%2896%2990729-X"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1159%2F000246792"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1016%2FS0190-9622%2889%2970123-7"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1365-2133.1990.tb06289.x"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1600-0560.1979.tb01131.x"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1016%2FS0190-9622%2883%2980227-8"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1086%2F323704"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1159%2F000247491"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1365-2133.1986.tb02832.x"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1016%2FS0190-9622%2881%2980176-4"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1136%2Fjcp.51.2.169"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1365-2230.1988.tb00693.x"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1046%2Fj.1365-2133.2000.03379.x"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1016%2FS0002-9270%2802%2904305-8"}],"container-title":["Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/81487","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/81487","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T23:45:25Z","timestamp":1745451925000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000081487"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005]]},"references-count":19,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2004,12,1]]}},"URL":"https:\/\/doi.org\/10.1159\/000081487","archive":["Portico"],"relation":{},"ISSN":["1018-8665","1421-9832"],"issn-type":[{"value":"1018-8665","type":"print"},{"value":"1421-9832","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005]]}}}