The Pericardium Cells Junctions Are a Target for Autoantibodies of Patients Affected by a Variant of Endemic Pemphigus Foliaceus in El Bagre and Surrounding Municipalities in Colombia, South America
Abstract
:1. Introduction
2. Materials and Methods
2.1. Patients
2.2. Clinical Evaluation
2.3. Histopathology (H&E Stain), Direct Immunofluorescence (DIF), Indirect Immunofluorescence (IIF), Confocal Microscopy (CM) and Immunohistochemical (IHC) Studies
2.4. Statistical Analysis
3. Results
3.1. Clinical Symptoms of the El Bagre-EPF Patients
3.2. Results of Immunohistochemistry for Patient Tissues (IHC)
3.3. Results of Immunofluorescence Tests (DIF and IIF) and Confocal Microscopy (CM)
4. Discussion
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
Abbreviations
EPF | Endemic pemphigus foliaceus |
El Bagre-EPF | Endemic pemphigus foliaceus in El Bagre |
PF | Pemphigus foliaceus |
FS | Fogo selvagem |
H&E | Hematoxylin and eosin |
DIF, IIF | Direct and indirect immunofluorescence |
BMZ | Base membrane zone |
ICS | Intercellular stain between keratinocytes |
PAF | Paraformaldehyde |
FITC | Fluorescein isothiocyanate |
DAPI | 4′,6-diamidino-2-phenylindole |
Dsg1 | Desmoglein 1 |
CFM | Confocal microscopy |
P0071 | Plakophilin 4 |
ARVCF | Armadillo repeat gene deleted in Velo-Cardio-Facial syndrome |
DP I-II | Desmoplakins I and II |
MYZAP | Myocardium-enriched zonula occludens-1-associated protein |
Abs | Antibodies |
ARVCF | Arrhythmogenic right ventricular cardiomyopathy |
ECG | Electrocardiogram |
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Catalogue Number | IHC All the Products from Agilent-Dako | E/P1 P | SNF2 P | E/P C | SNF Cs |
---|---|---|---|---|---|
AR0423 | Polyclonal rabbit anti-human IgG (Range 1:250 to 1:500) | (+++) | (+++) | (−) | (−) |
IR513 | Polyclonal rabbit anti-human IgM (Range 1:150 to 1:300). Flex ready to use. | (+++) | (+++) | (−) | (−) |
IR510 | Polyclonal rabbit anti-human IgA (Range 1:100 to 1:200). Flex ready to use. High Ph antigen retrieval. | (−) | (−) | (−) | (−) |
IR517 | Polyclonal rabbit anti-human IgD Flex ready to use. | (++) | (++) | (−) | (−) |
A0094 | Polyclonal rabbit anti-human IgE. (Range 1:750 to 1:1500). | (−) | (−) | (−) | (−) |
A 0062 | Polyclonal Rabbit anti-human C3C complement (Range 1:100 to 1:200). | (+++) | (+++) | (−) | (−) |
M077 | Monoclonal mouse anti-human C5b-9 complement (Range 1:25 to 1:50). (MAC). | (+++) | (+++) | (−) | (−) |
A0063 | Polyclonal rabbit anti-human C3d- complement (Range 1:400 to 1:800). | (+++) | (+++) | (−) | (−) |
A0136 | Polyclonal rabbit C1-q Complement (range not provided). | (++) | (++) | (−) | (−) |
A0001 | Polyclonal rabbit anti-human Albumin. (Range 1:2000 to 1:4000). | (++++) | (++++) | (−) | (−) |
A0080 | Polyclonal rabbit anti-human fibrinogen (Range 1:200 to 1:400). | (++++) | (++++) | (−) | (−) |
IR507 | Polyclonal rabbit anti-human Lambda. Flex ready to use. Heat induced epitope retrieval | (++++) | (++++) | (−) | (−) |
IR506 | Polyclonal rabbit anti-human Kappa light chains Flex ready to use. Heat induced epitope retrieval | (++++) | (++++) | (−) | (−) |
GA60066-2 | Negative control. Rabbit, FLEX RTU. Control reagent, Ready-to-use, Unconjugated for Immunohistochemistry, | (−) | (−) | (−) | (−) |
Symptoms/Signs, EKG/Chest RX | Patients | Controls |
---|---|---|
Sharp piercing chest pain over the center or left side of the chest, which is generally more intense when breathing. | 33/45 | 30/45 |
Continuous dyspnea. | 7/45 | 0/45 |
Pericardial rub on auscultation. | 2/45 | 0/45 |
Hiccups. | 7/45 | 2/45 |
Dysphagia. | 10/45 | 3/45 |
Hoarseness and syncope without respiratory infections. | 7/45 | 2/45 |
Current smokers. | 2/45 | 345 |
Past smokers. | 8/45 | 9/45 |
Palpitations. | 38/45 | 12/45 |
Fatigue. | 45/45 | 16/45 |
Weakness. | 39/45 | 1/45 |
Shortness of breath when reclining. | 7/45 | 0/45 |
Previous history of viral infections previous the above symptoms. | 45/45 | 45/45 |
Abdominal or leg swelling. | 7/45 | 1/45 |
Cardiomegaly using chest X Ray. | 19/45 | 3/45 |
New widespread ST-elevation or PR depression on ECG | 12/45 | 1/45 |
Pleural infusion by X-ray | 0/45 | 0/45 |
TEST Antigen Source Cow | P-Ab Sensory Nerve Formations at E/P (45) | P-Ab Functions at E/P (45) | Patients-Ab to Neural Epidermal Branches at E/P (45) | CM P Sensory Nerve Formations at EP (45) | Controls (45) |
---|---|---|---|---|---|
IIF IgG total | 43 | 43 | 43 | 43 | 0 |
IIF IgE | 1 | 1 | 1 | 1 | 0 |
IIF IgM | 42 | 42 | 42 | 42 | 0 |
IIF IgA | 0 | 0 | 0 | 0 | 0 |
IIF IgD | 25 | 25 | 25 | 25 | 0 |
IIF C3C | 38 | 38 | 38 | 38 | 0 |
IIF C1q | 35 | 35 | 35 | 35 | 0 |
IIF Fibrinogen | 45 | 45 | 45 | 45 | 0 |
IIF Albumin | 42 | 42 | 42 | 42 | 0 |
IIF Lambda | 45 | 45 | 45 | 45 | 0 |
IIF Kappa | 45 | 45 | 45 | 45 | 0 |
Confocal IIF IgG total | 43 | 43 | 43 | 43 | 0 |
Confocal IIF IgE | 1 | 1 | 1 | 1 | 0 |
Confocal IIF IgM | 42 | 42 | 42 | 42 | 0 |
Confocal IIF IgA | 0 | 0 | 0 | 0 | 0 |
Confocal IIF IgD | 25 | 25 | 25 | 25 | 0 |
Confocal IIF C3C | 38 | 38 | 38 | 38 | 0 |
Confocal IIF C1q | 35 | 35 | 35 | 35 | 0 |
Confocal IIF Fibrinogen | 45 | 45 | 45 | 45 | 0 |
Confocal IIF Albumin | 42 | 42 | 42 | 42 | 0 |
Confocal IIF lambda | 45 | 45 | 45 | 45 | 0 |
Confocal IIF Kappa | 45 | 45 | 45 | 45 | 0 |
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Abreu Velez, A.M.; Hashimoto, T.; Upegui, Y.A.; Vélez Arango, J.M.; Olarte Aponte, A.M.; Vega, J.A.; Howard, M.S. The Pericardium Cells Junctions Are a Target for Autoantibodies of Patients Affected by a Variant of Endemic Pemphigus Foliaceus in El Bagre and Surrounding Municipalities in Colombia, South America. Diagnostics 2025, 15, 964. https://doi.org/10.3390/diagnostics15080964
Abreu Velez AM, Hashimoto T, Upegui YA, Vélez Arango JM, Olarte Aponte AM, Vega JA, Howard MS. The Pericardium Cells Junctions Are a Target for Autoantibodies of Patients Affected by a Variant of Endemic Pemphigus Foliaceus in El Bagre and Surrounding Municipalities in Colombia, South America. Diagnostics. 2025; 15(8):964. https://doi.org/10.3390/diagnostics15080964
Chicago/Turabian StyleAbreu Velez, Ana Maria, Takashi Hashimoto, Yulieth A. Upegui, Jorge Mario Vélez Arango, Adriana Milena Olarte Aponte, Jose A. Vega, and Michael S. Howard. 2025. "The Pericardium Cells Junctions Are a Target for Autoantibodies of Patients Affected by a Variant of Endemic Pemphigus Foliaceus in El Bagre and Surrounding Municipalities in Colombia, South America" Diagnostics 15, no. 8: 964. https://doi.org/10.3390/diagnostics15080964
APA StyleAbreu Velez, A. M., Hashimoto, T., Upegui, Y. A., Vélez Arango, J. M., Olarte Aponte, A. M., Vega, J. A., & Howard, M. S. (2025). The Pericardium Cells Junctions Are a Target for Autoantibodies of Patients Affected by a Variant of Endemic Pemphigus Foliaceus in El Bagre and Surrounding Municipalities in Colombia, South America. Diagnostics, 15(8), 964. https://doi.org/10.3390/diagnostics15080964