{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,1,31]],"date-time":"2023-01-31T02:35:20Z","timestamp":1675132520810},"reference-count":33,"publisher":"Akademiai Kiado Zrt.","issue":"21","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2007,5,1]]},"abstract":"<jats:p>A paraneoplasticus pemphigus malignus vagy benignus tumorokhoz t\u00e1rsul\u00f3 autoimmun h\u00f3lyagos megbeteged\u00e9s diagnosztikus \u00e9s immunol\u00f3giai krit\u00e9riumait 1990-ben fektette le \n <jats:italic>Anhalt<\/jats:italic>\n . Klinikailag s\u00falyos, f\u00e1jdalmas, m\u00e9lyre terjed\u0151 b\u0151r- \u00e9s ny\u00e1lkah\u00e1rtyat\u00fcnetek jellemzik. A b\u0151rt\u00fcnetek polimorf jelleg\u0171ek, \u00e1ltal\u00e1ban domin\u00e1l a h\u00f3lyagk\u00e9pz\u0151d\u00e9s. Egyes esetekben, az \u00fan. \u201egraft-versus-host-disease\u201d form\u00e1kban el\u0151fordul, hogy kiz\u00e1r\u00f3lag papulosus, lichenoid b\u0151rt\u00fcnetek \u00e9szlelhet\u0151k, h\u00f3lyagk\u00e9pz\u0151d\u00e9s nincs, vagy csak k\u00e9s\u0151bb jelenik meg. Els\u0151sorban ezen alcsoport betegeiben a b\u0151r- \u00e9s ny\u00e1lkah\u00e1rtyat\u00fcnetek mellett s\u00falyos dyspno\u00e9t okoz\u00f3 pulmonalis \u00e9rintetts\u00e9g is kialakul, melynek h\u00e1tter\u00e9ben bronchiolitis obliterans \u00e1ll. A paraneoplasticus pemphigus diagnosztik\u00e1j\u00e1ban alapvet\u0151 fontoss\u00e1g\u00fa a b\u0151r\/ny\u00e1lkah\u00e1rty\u00e1k direkt immunfluoreszcens, tov\u00e1bb\u00e1 a sz\u00e9rum indirekt immunhisztol\u00f3giai \u00e9s immunoblot vizsg\u00e1lata. Az eddig azonos\u00edtott autoantig\u00e9nek d\u00f6nt\u0151 t\u00f6bbs\u00e9ge a plakincsal\u00e1d tagja: envoplakin (210 kDa), periplakin (190 kDa), plectin (~500 kDa), desmoplakin I (250 kDa), desmoplakin II (210 kDa), bullosus pemphigoid antig\u00e9n 1 (230 kDa). A plakinok mellett a desmosomalis cadherinek k\u00f6z\u00e9 sorolt desmoglein 1 \u00e9s 3 a bullosus pemphigoid antig\u00e9n 2 (180 kDa), a desmocollin 2 \u00e9s 3, tov\u00e1bb\u00e1 egy m\u00e9g nem azonos\u00edtott, 170 kDa molekulat\u00f6meg\u0171 transzmembr\u00e1n feh\u00e9rje szint\u00e9n autoantig\u00e9nje a k\u00f3rk\u00e9pnek. A paraneoplasticus pemphigus nagy mortalit\u00e1s\u00fa k\u00f3rk\u00e9p, az esetek t\u00f6bb mint 90%-\u00e1ban hal\u00e1los kimenetel\u0171. A h\u00e1tt\u00e9rben \u00e1ll\u00f3 tumor elt\u00e1vol\u00edt\u00e1sa mellett a b\u00e1zister\u00e1pia tov\u00e1bbra is a nagy d\u00f3zis\u00fa sziszt\u00e9m\u00e1s szteroidkezel\u00e9s, melyet citosztatikumok, immunmodul\u00e1nsok ad\u00e1s\u00e1val eg\u00e9sz\u00edtenek ki. Sz\u00f3ba j\u00f6n ezenk\u00edv\u00fcl plasmapheresis, plazmacsere, photopheresis, nagy d\u00f3zis\u00fa intrav\u00e9n\u00e1s immunglobulin \u00e9s anti-CD20 monoklon\u00e1lis antitest (rituximab) ad\u00e1sa is.<\/jats:p>","DOI":"10.1556\/oh.2007.27955","type":"journal-article","created":{"date-parts":[[2007,5,23]],"date-time":"2007-05-23T15:18:30Z","timestamp":1179933510000},"page":"979-983","source":"Crossref","is-referenced-by-count":7,"title":["Paraneoplastic pemphigus"],"prefix":"10.1556","volume":"148","author":[{"given":"Klaudia","family":"Preisz","sequence":"first","affiliation":[{"name":"1 Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar B\u0151r-, Nemik\u00f3rtani \u00e9s B\u0151ronkol\u00f3giai Klinika Budapest M\u00e1ria u. 41. 1085"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sarolta","family":"K\u00e1rp\u00e1ti","sequence":"additional","affiliation":[{"name":"1 Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar B\u0151r-, Nemik\u00f3rtani \u00e9s B\u0151ronkol\u00f3giai Klinika Budapest M\u00e1ria u. 41. 1085"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"425","reference":[{"key":"atypb1","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1172\/JCI3647","volume":"102","year":"1998","journal-title":"J. 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