Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus

Authors: Wang, J.; Bu, D.F.; Li, T.1; Zheng, R.; Zhang, B.X.; Chen, X.X.; Zhu, X.J.

Source: British Journal of Dermatology, Volume 153, Number 3, September 2005 , pp. 558-564(7)

Publisher: Blackwell Publishing

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Abstract:

Summary Background

Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease. We previously reported that B cells in a Castleman tumour associated with PNP produced autoantibodies. However, it is uncertain whether the production of autoantibodies from the associated tumour is a common mechanism in PNP. Objectives

To investigate autoantibody production in a thymoma and a follicular dendritic cell sarcoma that were excised from two patients with PNP. Methods

Tumour cells were cultured, and their surface markers were identified. Indirect immunofluorescence, immunoblotting and enzyme-linked immunosorbent assay (ELISA) using culture media from the tumours were used to detect PNP autoantibodies. Results

B cells with markers (CD22+, surface membrane IgG+ and surface membrane IgM+) of mature B lymphocytes constituted a proportion of cultured tumour cells in both tumours. Western blot showed that the medium from both the thymoma and the follicular dendritic cell sarcoma cells recognized 190-kDa periplakin and 210-kDa envoplakin bands of human epithelial proteins as well as recombinant linker regions of periplakin, envoplakin, desmoplakin and bullous pemphigoid antigen 1. ELISA was positive for antidesmoglein 3 antibody. Conclusions

The presence and localization in tumours of B-lymphocyte clones against proteins of the plakin family and desmoglein 3 in skin may not be confined to PNP with Castleman disease, but is possibly a common mechanism in PNP associated with various tumours.

Keywords: autoantibody; follicular dendritic cell sarcoma; paraneoplastic pemphigus; thymoma

Document Type: Research article

DOI: 10.1111/j.1365-2133.2005.06599.x

Affiliations: 1: Pathology, Peking University First Hospital, 8 Xishiku St, Beijing 100034, China

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