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具有获得性大疱性表皮松解症表型的副肿瘤性免疫性大疱病:两例经妇科恶性肿瘤治疗后缓解的病例

Paraneoplastic immunobullous disease with an epidermolysis bullosa acquisita phenotype: two cases demonstrating remission with treatment of gynaecological malignancy.

作者信息

Chamberlain Alexander J, Cooper Susan M, Allen Jill, Dean Diane, Baxter Kay F, Goodfield Mark J D, Wojnarowska Fenella

机构信息

Department of Dermatology, The Churchill, Oxford Radcliffe Hospitals, Oxford, UK.

出版信息

Australas J Dermatol. 2004 May;45(2):136-9. doi: 10.1111/j.1440-0960.2004.00068.x.

Abstract

Two cases of paraneoplastic immunobullous disease occurring in women with gynaecological malignancies are reported. Both cases demonstrated mechanobullous mucocutaneous blistering as is typically seen in epidermolysis bullosa acquisita. Their immunopathology, however, favoured a dermal-binding mucous membrane pemphigoid (MMP) (or possibly bullous pemphigoid) for patient 1 and laminin-5 MMP for patient 2. Both patients showed resolution of blistering within 1 year of treatment of their malignancies; uterine and ovarian carcinoma, respectively. These cases are of interest because of their paraneoplastic nature; as well as overlapping clinicoimmunopathological features. In addition, patient 2 is, as far as we are aware, the first report of ovarian-carcinoma-associated laminin-5 MMP.

摘要

本文报告了两例发生于妇科恶性肿瘤女性患者的副肿瘤性免疫性大疱病。两例患者均表现为机械性大疱性黏膜皮肤水疱,这是获得性大疱性表皮松解症的典型表现。然而,其免疫病理学检查结果显示,病例1倾向于诊断为真皮结合型黏膜类天疱疮(MMP)(或可能为大疱性类天疱疮),病例2则倾向于诊断为层粘连蛋白-5 MMP。两名患者在分别接受子宫癌和卵巢癌治疗后的1年内,水疱均消退。这些病例因其副肿瘤性质以及重叠的临床免疫病理特征而备受关注。此外,据我们所知,病例2是卵巢癌相关层粘连蛋白-5 MMP的首例报告。

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