Melnick Laura E, Beasley Jenna M, Kim Randi, Brinster Nooshin, Sicco Kristen Lo
New York University Health, New York.
Dermatol Online J. 2017 Dec 15;23(12):13030/qt8sf4c5xn.
Paraneoplastic Pemphigus (PNP) is a rare and often fatal autoimmune mucocutaneous blistering disease associated with an underlying malignancy. It is thought to be caused by antibodies to tumor antigenscross-reacting with epithelial antigens, specifically desmosomal and hemidesmosomal antigens. There are at least five clinical morphologic variants of PNP, with the earliest and most consistent finding beingsevere stomatitis. Diagnosis of PNP requires direct immunofluorescence of perilesional skin and indirect immunofluorescence. Treatment of PNP is difficult and largely limited to glucocorticoids, steroid-sparing immunomodulators, rituximab and intravenous immunoglobulin (IVIG). Despite therapies, prognosis is poor. We report a case of paraneoplastic pemphigus in a 34-year old male with severe stomatitis and lichenplanus-like cutaneous lesions.
副肿瘤性天疱疮（PNP）是一种罕见且常致命的自身免疫性黏膜皮肤水疱病，与潜在的恶性肿瘤相关。它被认为是由针对肿瘤抗原的抗体与上皮抗原发生交叉反应引起的，特别是桥粒和半桥粒抗原。PNP至少有五种临床形态学变体，最早且最一致的表现是严重的口腔炎。PNP的诊断需要对皮损周围皮肤进行直接免疫荧光检查和间接免疫荧光检查。PNP的治疗困难，主要限于糖皮质激素、糖皮质激素节省免疫调节剂、利妥昔单抗和静脉注射免疫球蛋白（IVIG）。尽管有多种治疗方法，但预后较差。我们报告一例34岁男性的副肿瘤性天疱疮病例，该患者有严重的口腔炎和扁平苔藓样皮肤损害。
