{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2022,10,26]],"date-time":"2022-10-26T04:23:43Z","timestamp":1666758223882},"reference-count":0,"publisher":"Ordem dos Medicos","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Med Port"],"abstract":"<jats:p>A elastose perfurante serpiginosa \u00e9 uma dermatose perfurante rara, encontrada sobretudo em adolescentes e adultos jovens, caraterizada pela elimina\u00e7\u00e3o transepid\u00e9rmica de fibras el\u00e1sticas an\u00f3malas. O \u00fanico f\u00e1rmaco conhecido capaz de induzir elastose perfurante serpiginosa \u00e9 a D-penicilamina. Descrevemos o caso de uma doente de 52 anos com p\u00e1pulas querat\u00f3sicas confluentes com disposi\u00e7\u00e3o anular e crescimento centr\u00edfugo, localizadas na regi\u00e3o cervical anterior. A doente estava cronicamente medicada com D-penicilamina, por doen\u00e7a de Wilson. A bi\u00f3psia lesional revelou elimina\u00e7\u00e3o transepid\u00e9rmica de fibras el\u00e1sticas com aumento da eosinofilia, espessadas, ramificadas e de aspeto em dentes de serra. Os achados clinicopatol\u00f3gicos foram compat\u00edveis com elastose perfurante serpiginosa secund\u00e1ria \u00e0 D-penicilamina. Estima-se que a elastose perfurante serpiginosa ocorra em 1% dos doentes medicados com D-penicilamina. Bloqueando direta ou indiretamente as liga\u00e7\u00f5es cruzadas de desmosina da elastina, a D-penicilamina leva \u00e0 s\u00edntese de fibras el\u00e1sticas an\u00f3malas d\u00e9rmicas, mas tamb\u00e9m extracut\u00e2neas. A elastose perfurante serpiginosa pode ser a primeira manifesta\u00e7\u00e3o de um processo degenerativo multissist\u00e9mico do tecido conjuntivo el\u00e1stico.<\/jats:p>","DOI":"10.20344\/amp.6749","type":"journal-article","created":{"date-parts":[[2016,7,2]],"date-time":"2016-07-02T07:42:43Z","timestamp":1467445363000},"page":"227-230","source":"Crossref","is-referenced-by-count":2,"title":["Elastose Perfurante Serpiginosa e Doen\u00e7a de Wilson: Uma Consequ\u00eancia Rara, mas Previs\u00edvel da Terap\u00eautica a Longo Prazo com D-Penicilamina"],"prefix":"10.20344","volume":"29","author":[{"given":"Andr\u00e9 Castro","family":"Pinho","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jos\u00e9 Carlos","family":"Cardoso","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miguel","family":"Gouveia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hugo","family":"Oliveira","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"8464","published-online":{"date-parts":[[2016,3,31]]},"container-title":["Acta M\u00e9dica Portuguesa"],"original-title":[],"link":[{"URL":"https:\/\/www.actamedicaportuguesa.com\/revista\/index.php\/amp\/article\/download\/6749\/4623","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.actamedicaportuguesa.com\/revista\/index.php\/amp\/article\/download\/6749\/4623","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,10,25]],"date-time":"2022-10-25T21:47:37Z","timestamp":1666734457000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.actamedicaportuguesa.com\/revista\/index.php\/amp\/article\/view\/6749"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,3,31]]},"references-count":0,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2016,3,31]]}},"URL":"https:\/\/doi.org\/10.20344\/amp.6749","relation":{},"ISSN":["1646-0758","0870-399X"],"issn-type":[{"value":"1646-0758","type":"electronic"},{"value":"0870-399X","type":"print"}],"subject":[],"published":{"date-parts":[[2016,3,31]]}}}