Unit of Dermatology and Cosmetology, IRCCS University Vita-Salute San Raffaele, 20132 Milan, Italy.
Dipartimento di Medicina Interna e Specialità Mediche, Dermatologia, Sapienza Università di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy.
Int J Mol Sci. 2017 Nov 26;18(12):2532. doi: 10.3390/ijms18122532.
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.
副肿瘤性天疱疮是一种罕见的自身免疫性皮肤病，总是与肿瘤有关。通常，副肿瘤性天疱疮患者最早表现为口腔、皮肤和黏膜损伤。副肿瘤性天疱疮的发病机制尚未完全阐明，尽管最近已经阐明了一些免疫学方面。由于其罕见性，已经提出了几种诊断标准。此外，还使用了几种诊断程序进行诊断，包括间接免疫荧光、直接免疫荧光和 ELISA。我们查阅了最近的文献，在 PubMed 上搜索了“副肿瘤性天疱疮”。我们还包括了法语、德语和西班牙语的论文。我们找到了 613 篇关于“副肿瘤性天疱疮”的论文。其中，有 169 篇是综述。由于其不同的临床特征，副肿瘤性天疱疮仍然是临床医生面临的挑战。此外，副肿瘤性天疱疮的诊断和治疗需要皮肤科医生、肿瘤学家和耳鼻喉科医生之间的密切合作。
