Antibodies to Laminin β4 in Pemphigoid Diseases: Clinical–Laboratory Experience of a Single Central European Reference Centre
Abstract
1. Introduction
2. Materials and Methods
2.1. Direct Immunofluorescence Procedure
2.2. Multiplex ELISA Procedure
2.3. Mosaic Laminin β4 Indirect Immunofluorescence Procedure
3. Results
4. Discussion
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Patient | Gender/Age (Years) | DIF Findings | IIF for Laminin β4 | Clinical Presentation |
---|---|---|---|---|
1 | F/82 | Linear deposits of IgG1 (+), IgG4 (++), and C3 (++) along the DEJ | 1:100 | Predominantly acral vesicles and blisters in an erythema multiforme-like arrangement |
2 | M/61 | Linear deposits of IgG4 (++) along the DEJ | 1:10 | Disseminated papulopustular, folliculitis-like lesions on the trunk, back, and extremities |
3 | F/78 | Linear deposits of IgG1 (+), IgG4 (+++), and C3 (+++) along the DEJ | 1:10 | Tense blisters and erosions distributed across the entire body, including acral regions |
4 | M/93 | Linear deposits of IgG1 (+), IgG4 (++), and C3 (+++) along the DEJ | 1:10 | Urticarial plaques predominantly on the trunk and lateral aspects of the thighs |
5 | M/71 | Linear deposits of IgG4 (++) and C3 (+++) along the DEJ | 1:10 | Disseminated erosions covered with brownish crusts, mainly on flexural aspects of the limbs and soles of the feet |
6 | M/89 | Linear deposits of IgM (+), IgG1 (+), IgG4 (++), and C3 (+++) along the DEJ | 1:100 | Acral blisters and urticarial lesions on the scrotum and medial aspects of the thighs |
7 | F/68 | Linear deposits of IgG1 (+) and C3 (+++) along the DEJ | Negative | Erosions with crusts predominantly on the scalp; few erosions on the trunk |
8 | F/90 | Linear deposits of IgG1 (++) and C3 (+++) along the DEJ | Negative | Isolated erosions on the scalp, with multiple erosions primarily on the trunk and to a lesser extent on the upper extremities |
9 | F/91 | Linear deposits of IgG1 (+/−), IgG4 (+), and C3 (++) along the DEJ | Negative | Disseminated, well-tensed blisters on the trunk and extremities, with scattered erosions |
10 | M/67 | Linear deposits of C3 (++) along the DEJ | Negative | Erythematous and erosive lesions with well-tensed blisters on the extremities, and to a lesser extent on the trunk |
Patient | Gender/Age (Years) | DIF Findings | ELISA Results (BP180 and BP230) | IIF for Laminin β4 |
---|---|---|---|---|
1 | F/70 | Linear deposits of C3 (+++) along the DEJ | BP180: 7.54; BP230: 3.42 | Negative |
2 | F/81 | Linear deposits of IgG4 (+) and C3 (++) along the DEJ | BP180: 9.82; BP230: 5.82 | Negative |
3 | M/80 | Linear deposits of IgG1 (+), IgG4 (++), and C3 (++) along the DEJ | BP180: 1.02 | Negative |
4 | F/78 | Linear deposits of IgG4 (+) and C3 (+++) along the DEJ | BP180: 10.72 | Negative |
5 | M/86 | Linear deposits of IgG4 (+) and C3 (++) along the DEJ | BP180: 11.44 | Negative |
6 | M/71 | Linear deposits of IgG4 (+) and C3 (++) along the DEJ | BP180: 8.33 | Negative |
7 | M/80 | Linear deposits of IgG (+), IgG1 (+++), IgG4 (+), and C3 (+) along the DEJ | BP180: 7.03 | Negative |
8 | F/80 | Linear deposits of IgG4 (++) and C3 (++) along the DEJ | BP180: 7.19 | Negative |
9 | M/73 | Linear deposits of C3 (+++) along the DEJ | BP180: 8.03 | Negative |
10 | F/85 | Linear deposits of IgG (+), IgG4 (+), and C3 (++) along the DEJ | BP180: 5.35; BP230: 1.29 | Negative |
11 | F/83 | Linear deposits of IgG (+), IgG1 (+), IgG4 (+), and C3 (++) along the DEJ | BP180: 8.95; BP230: 5.00 | Negative |
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Spałek, M.M.; Jałowska, M.; Welc, N.; Bowszyc-Dmochowska, M.; Hashimoto, T.; Gornowicz-Porowska, J.; Dmochowski, M. Antibodies to Laminin β4 in Pemphigoid Diseases: Clinical–Laboratory Experience of a Single Central European Reference Centre. Antibodies 2025, 14, 66. https://doi.org/10.3390/antib14030066
Spałek MM, Jałowska M, Welc N, Bowszyc-Dmochowska M, Hashimoto T, Gornowicz-Porowska J, Dmochowski M. Antibodies to Laminin β4 in Pemphigoid Diseases: Clinical–Laboratory Experience of a Single Central European Reference Centre. Antibodies. 2025; 14(3):66. https://doi.org/10.3390/antib14030066
Chicago/Turabian StyleSpałek, Maciej Marek, Magdalena Jałowska, Natalia Welc, Monika Bowszyc-Dmochowska, Takashi Hashimoto, Justyna Gornowicz-Porowska, and Marian Dmochowski. 2025. "Antibodies to Laminin β4 in Pemphigoid Diseases: Clinical–Laboratory Experience of a Single Central European Reference Centre" Antibodies 14, no. 3: 66. https://doi.org/10.3390/antib14030066
APA StyleSpałek, M. M., Jałowska, M., Welc, N., Bowszyc-Dmochowska, M., Hashimoto, T., Gornowicz-Porowska, J., & Dmochowski, M. (2025). Antibodies to Laminin β4 in Pemphigoid Diseases: Clinical–Laboratory Experience of a Single Central European Reference Centre. Antibodies, 14(3), 66. https://doi.org/10.3390/antib14030066