一例与慢性淋巴细胞白血病相关的IgA副肿瘤性天疱疮的新病例。

A novel case of IgA paraneoplastic pemphigus associated with chronic lymphocytic leukemia.

作者信息

Taintor Adam R, Leiferman Kristin M, Hashimoto Takashi, Ishii Norito, Zone John J, Hull Christopher M

机构信息

Department of Dermatology, University of Utah School of Medicine, Salt Lake City, UT 84132, USA.

出版信息

J Am Acad Dermatol. 2007 May;56(5 Suppl):S73-6. doi: 10.1016/j.jaad.2006.11.023.

DOI:10.1016/j.jaad.2006.11.023

PMID:17434044

Abstract

Paraneoplastic pemphigus is an autoimmune vesiculobullous and erosive mucocutaneous disease associated with an underlying malignancy. Reported malignancies include chronic lymphocytic leukemia, non-Hodgkin's lymphoma, Castleman's disease, sarcomas, and rarely solid tumors. Patients with paraneoplastic pemphigus develop characteristic IgG autoantibodies against several antigens including members of the plakin family, bullous pemphigoid antigen 1, and desmosomal proteins. IgA pemphigus is another recently characterized immunobullous disease that presents as a vesiculopustular eruption with neutrophilic infiltration and epidermal acantholysis. Mucous membrane involvement is rare. We report what is to our knowledge a unique case with features of both IgA pemphigus and paraneoplastic pemphigus associated with chronic lymphocytic leukemia.

摘要

副肿瘤性天疱疮是一种自身免疫性水疱大疱性和糜烂性黏膜皮肤疾病，与潜在的恶性肿瘤相关。报告的恶性肿瘤包括慢性淋巴细胞白血病、非霍奇金淋巴瘤、Castleman病、肉瘤，以及罕见的实体瘤。副肿瘤性天疱疮患者会产生针对多种抗原的特征性IgG自身抗体，这些抗原包括桥粒斑蛋白家族成员、大疱性类天疱疮抗原1和桥粒蛋白。IgA天疱疮是另一种最近被描述的免疫性大疱性疾病，表现为伴有嗜中性粒细胞浸润和表皮棘层松解的水疱脓疱性皮疹。黏膜受累罕见。我们报告了据我们所知的一例独特病例，其具有与慢性淋巴细胞白血病相关的IgA天疱疮和副肿瘤性天疱疮的特征。