Kennedy N A, Dawe S
Flinders Medical Centre, SA, Australia.
Clin Exp Dermatol. 2009 Jul;34(5):e130-3. doi: 10.1111/j.1365-2230.2008.03150.x. Epub 2009 May 5.
Paraneoplastic pemphigus (PNP) is a mucocutaneous immunobullous disease associated with several types of internal malignancy. We report a case of a 78-year-old woman presenting with an atypical form of PNP associated with a recurrence of endometrial cancer. There was no involvement of the mucous membranes. Although the macroscopic and histological appearances were typical for IgA pemphigus or Sneddon-Wilkinson disease, direct immunofluorescence was positive for intercellular IgG and C3 staining. Circulating antibodies were detected to periplakin and envoplakin, but not to desmogleins 1 or 3. This case therefore meets the modified criteria for PNP. A response was seen to a combination of topical and systemic corticosteroids and acitretin, although with cyclical recurrences. These ceased after resection of her recurrent endometrial carcinoma.
副肿瘤性天疱疮（PNP）是一种与多种类型的内部恶性肿瘤相关的黏膜皮肤免疫性大疱性疾病。我们报告一例78岁女性，患有与子宫内膜癌复发相关的非典型PNP。未累及黏膜。尽管大体和组织学表现符合IgA天疱疮或Sneddon-Wilkinson病，但直接免疫荧光显示细胞间IgG和C3染色呈阳性。检测到循环抗体针对周膜蛋白和内披蛋白，但不针对桥粒芯糖蛋白1或3。因此，该病例符合PNP的修订标准。局部和全身使用皮质类固醇及阿维A联合治疗有效，尽管有周期性复发。在切除复发性子宫内膜癌后复发停止。
