University of Oulu

Nätynki A, Tuusa J, Hervonen K, Kaukinen K, Lindgren O, Huilaja L, Kokkonen N, Salmi T and Tasanen K (2020) Autoantibodies Against the Immunodominant Bullous Pemphigoid Epitopes Are Rare in Patients With Dermatitis Herpetiformis and Coeliac Disease. Front. Immunol. 11:575805. doi: 10.3389/fimmu.2020.575805

Autoantibodies against the immunodominant bullous pemphigoid epitopes are rare in patients with dermatitis herpetiformis and coeliac disease

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Author: Nätynki, Antti1; Tuusa, Jussi1; Hervonen, Kaisa2,3;
Organizations: 1PEDEGO Research Unit, Department of Dermatology, Medical Research Center Oulu, Oulu University Hospital, University of Oulu, Oulu, Finland
2Department of Dermatology, Tampere University Hospital, Tampere, Finland
3Celiac Disease Research Center, Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland
4Department of Internal Medicine, Tampere University Hospital, Tampere, Finland
5Department of Pathology, Medical Research Center Oulu, Oulu University Hospital, University of Oulu, Oulu, Finland
Format: article
Version: published version
Access: open
Online Access: PDF Full Text (PDF, 1.4 MB)
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Language: English
Published: Frontiers Media, 2020
Publish Date: 2021-02-15


Dermatitis herpetiformis (DH) is an extraintestinal manifestation of coeliac disease (CD). Patients with DH have an elevated risk of development of another autoimmune blistering skin disease, bullous pemphigoid (BP). In this study we investigated whether patients with DH and CD (mean age for both 49 years) have circulating autoantibodies against BP180, the major BP autoantigen. ELISA tests showed that only a few DH (3/46) and CD (2/43) patients had BP180-NC16A IgG autoantibodies. Immunoblotting found that more than half of the DH samples contained IgG autoantibodies against full-length BP180. Epitope mapping with 13 fusion proteins covering the BP180 polypeptide revealed that in DH and CD patients, IgG autoantibodies did not target the NC16A or other epitopes typical of BP but recognized other intracellular and mid-extracellular regions of BP180. None of the analyzed DH and CD patients with either ELISA or immunoblotting positivity had IgG or IgA reactivity against the cutaneous basement membrane in indirect immunofluorescence analysis or skin symptoms characteristic of BP. Although only a minority of middle-aged DH patients had IgG autoantibodies against the immunodominant epitopes of BP180, our results do not exclude the possibility that intermolecular epitope spreading could explain the switch from DH to BP in elderly patients.

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Series: Frontiers in immunology
ISSN: 1664-3224
ISSN-E: 1664-3224
ISSN-L: 1664-3224
Volume: 11
Article number: 575805
DOI: 10.3389/fimmu.2020.575805
Type of Publication: A1 Journal article – refereed
Field of Science: 3121 General medicine, internal medicine and other clinical medicine
Funding: This study was supported by research grants from the Academy of Finland, the Sigrid Juselius Foundation, the University of Oulu Graduate School, the Medical Research Center, Oulu University Hospital (MRC Oulu) and the Competitive State Research Financing of the Expert Area of Tampere University Hospital.
Copyright information: © 2020 Nätynki, Tuusa, Hervonen, Kaukinen, Lindgren, Huilaja, Kokkonen, Salmi and Tasanen. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.