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Int. J. Mol. Sci. 2017, 18(12), 2532; https://doi.org/10.3390/ijms18122532

Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy

1
Unit of Dermatology and Cosmetology, IRCCS University Vita-Salute San Raffaele, 20132 Milan, Italy
2
Dipartimento di Medicina Interna e Specialità Mediche, Dermatologia, Sapienza Università di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy
3
Prima Divisione di Dermatologia, Istituto Dermopatico dell’Immacolata-IRCCS, Via dei Monti di Creta, 104, 00167 Roma, Italy
4
Dipartimento di Organi di Senso, Sapienza Università di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy
5
Dipartimento di Anatomia Patologica, Università Campus-Biomedico, 00128 Roma, Italy
6
Dipartimento di Immunologia Clinica, Sapienza Università di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy
7
Dipartimento di Farmacologia, Sapienza Università di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy
*
Author to whom correspondence should be addressed.
Received: 28 September 2017 / Revised: 9 November 2017 / Accepted: 24 November 2017 / Published: 26 November 2017
(This article belongs to the Special Issue Inflammatory Skin Conditions 2017)
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Abstract

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 “paraneoplastic pemphigus”. We included also papers in French, German, and Spanish. We found 613 papers for “paraneoplastic pemphigus”. 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. View Full-Text
Keywords: oncology; oral lesions; paraneoplastic pemphigus; therapy oncology; oral lesions; paraneoplastic pemphigus; therapy
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Paolino, G.; Didona, D.; Magliulo, G.; Iannella, G.; Didona, B.; Mercuri, S.R.; Moliterni, E.; Donati, M.; Ciofalo, A.; Granata, G.; Ranuzzi, P.; Falasca, V.; Calvieri, S. Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy. Int. J. Mol. Sci. 2017, 18, 2532.

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