{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T18:19:23Z","timestamp":1762021163609,"version":"build-2065373602"},"reference-count":0,"publisher":"Ordem dos Medicos","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Med Port"],"abstract":"<jats:p>Introdu\u00e7\u00e3o: As mastocitoses caraterizam-se pela expans\u00e3o clonal de mast\u00f3citos, com acumula\u00e7\u00e3o de mast\u00f3citos morfol\u00f3gica e\u00a0imunofenotipicamente anormais em diferentes \u00f3rg\u00e3os. A pele \u00e9 o \u00f3rg\u00e3o mais frequentemente envolvido. Virtualmente, todas as\u00a0crian\u00e7as e mais de 80% dos adultos com mastocitose apresentam les\u00f5es cut\u00e2neas.Material e M\u00e9todos: O presente artigo descreve os sinais e sintomas associados \u00e0 mastocitose na pele, tendo por base a revis\u00e3o das\u00a0normas de orienta\u00e7\u00e3o de consenso internacionais, recentemente publicadas.Discuss\u00e3o: De acordo com a classifica\u00e7\u00e3o proposta pela Organiza\u00e7\u00e3o Mundial de Sa\u00fade em 2016, a mastocitose divide-se em\u00a0mastocitose cut\u00e2nea, mastocitose sist\u00e9mica e sarcoma de mast\u00f3citos. A mastocitose cut\u00e2nea pode subdividir-se em tr\u00eas subtipos: a\u00a0mastocitose cut\u00e2nea maculopapular (tamb\u00e9m denominada urtic\u00e1ria pigmentosa), mastocitose cut\u00e2nea difusa e mastocitoma cut\u00e2neo.\u00a0A telangiectasia macular eruptiva perstans j\u00e1 n\u00e3o \u00e9 considerada uma entidade independente.Conclus\u00e3o: As manifesta\u00e7\u00f5es cut\u00e2neas da mastocitose s\u00e3o vari\u00e1veis, dependendo da idade de in\u00edcio da doen\u00e7a. Recentemente\u00a0a classifica\u00e7\u00e3o da mastocitose cut\u00e2nea foi atualizada. Nas crian\u00e7as, a mastocitose ocorre como mastocitose cut\u00e2nea que tende \u00e0\u00a0regress\u00e3o espont\u00e2nea durante a adolesc\u00eancia. Quando tem in\u00edcio na idade adulta, a mastocitose \u00e9 geralmente sist\u00e9mica, sendo a\u00a0forma mais frequente a mastocitose sist\u00e9mica indolente, que normalmente tamb\u00e9m cursa com manifesta\u00e7\u00f5es cut\u00e2neas e tem um\u00a0curso cr\u00f3nico.<\/jats:p>","DOI":"10.20344\/amp.12189","type":"journal-article","created":{"date-parts":[[2020,4,1]],"date-time":"2020-04-01T13:14:48Z","timestamp":1585746888000},"page":"275-281","source":"Crossref","is-referenced-by-count":3,"title":["Manifesta\u00e7\u00f5es Cut\u00e2neas nas Mastocitoses: Atualiza\u00e7\u00e3o"],"prefix":"10.20344","volume":"33","author":[{"given":"Sandra","family":"Ferreira","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Iolanda","family":"Fernandes","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Renata","family":"Cabral","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susana","family":"Machado","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margarida","family":"Lima","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Manuela","family":"Selores","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"8464","published-online":{"date-parts":[[2020,4,1]]},"container-title":["Acta M\u00e9dica Portuguesa"],"original-title":[],"link":[{"URL":"https:\/\/www.actamedicaportuguesa.com\/revista\/index.php\/amp\/article\/download\/12189\/5910","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.actamedicaportuguesa.com\/revista\/index.php\/amp\/article\/download\/12189\/5910","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,10,24]],"date-time":"2022-10-24T19:55:35Z","timestamp":1666641335000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.actamedicaportuguesa.com\/revista\/index.php\/amp\/article\/view\/12189"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,4,1]]},"references-count":0,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2020,4,1]]}},"URL":"https:\/\/doi.org\/10.20344\/amp.12189","relation":{},"ISSN":["1646-0758","0870-399X"],"issn-type":[{"type":"electronic","value":"1646-0758"},{"type":"print","value":"0870-399X"}],"subject":[],"published":{"date-parts":[[2020,4,1]]}}}