{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T08:59:30Z","timestamp":1790499570402,"version":"4.1.0"},"reference-count":92,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2017,11,26]],"date-time":"2017-11-26T00:00:00Z","timestamp":1511654400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by paraneoplastic pemphigus patients. The pathogenesis of paraneoplastic pemphigus is not yet completely understood, although some immunological aspects have been recently clarified. Because of its rarity, several diagnostic criteria have been proposed. Besides, several diagnostic procedures have been used for the diagnosis, including indirect immunofluorescence, direct immunofluorescence, and ELISA. We reviewed the most recent literature, searching on PubMed \u201cparaneoplastic pemphigus\u201d. We included also papers in French, German, and Spanish. We found 613 papers for \u201cparaneoplastic pemphigus\u201d. Among them, 169 were review papers. Because of its varying clinical features, paraneoplastic pemphigus still represents a challenge for clinicians. Furthermore, diagnosis and management of paraneoplastic pemphigus requires close collaboration between physicians, including dermatologist, oncologist, and otorhinolaryngologist.<\/jats:p>","DOI":"10.3390\/ijms18122532","type":"journal-article","created":{"date-parts":[[2017,11,27]],"date-time":"2017-11-27T11:07:08Z","timestamp":1511780828000},"page":"2532","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":120,"title":["Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy"],"prefix":"10.3390","volume":"18","author":[{"given":"Giovanni","family":"Paolino","sequence":"first","affiliation":[{"name":"Unit of Dermatology and Cosmetology, IRCCS University Vita-Salute San Raffaele, 20132 Milan, Italy"},{"name":"Dipartimento di Medicina Interna e Specialit\u00e0 Mediche, Dermatologia, Sapienza Universit\u00e0 di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dario","family":"Didona","sequence":"additional","affiliation":[{"name":"Prima Divisione di Dermatologia, Istituto Dermopatico dell\u2019Immacolata-IRCCS, Via dei Monti di Creta, 104, 00167 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Magliulo","sequence":"additional","affiliation":[{"name":"Dipartimento di Organi di Senso, Sapienza Universit\u00e0 di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1781-2809","authenticated-orcid":false,"given":"Giannicola","family":"Iannella","sequence":"additional","affiliation":[{"name":"Dipartimento di Organi di Senso, Sapienza Universit\u00e0 di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Biagio","family":"Didona","sequence":"additional","affiliation":[{"name":"Prima Divisione di Dermatologia, Istituto Dermopatico dell\u2019Immacolata-IRCCS, Via dei Monti di Creta, 104, 00167 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Santo","family":"Mercuri","sequence":"additional","affiliation":[{"name":"Unit of Dermatology and Cosmetology, IRCCS University Vita-Salute San Raffaele, 20132 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Moliterni","sequence":"additional","affiliation":[{"name":"Dipartimento di Medicina Interna e Specialit\u00e0 Mediche, Dermatologia, Sapienza Universit\u00e0 di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele","family":"Donati","sequence":"additional","affiliation":[{"name":"Dipartimento di Anatomia Patologica, Universit\u00e0 Campus-Biomedico, 00128 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Ciofalo","sequence":"additional","affiliation":[{"name":"Dipartimento di Organi di Senso, Sapienza Universit\u00e0 di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guido","family":"Granata","sequence":"additional","affiliation":[{"name":"Dipartimento di Immunologia Clinica, Sapienza Universit\u00e0 di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Ranuzzi","sequence":"additional","affiliation":[{"name":"Dipartimento di Farmacologia, Sapienza Universit\u00e0 di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincenzo","family":"Falasca","sequence":"additional","affiliation":[{"name":"Dipartimento di Organi di Senso, Sapienza Universit\u00e0 di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Calvieri","sequence":"additional","affiliation":[{"name":"Dipartimento di Medicina Interna e Specialit\u00e0 Mediche, Dermatologia, Sapienza Universit\u00e0 di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2017,11,26]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1729","DOI":"10.1056\/NEJM199012203232503","article-title":"Paraneoplastic pemphigus. 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