Patients affected by El Bagre-EPF have autoantibodies to APM, colocalizing with the antibodies MYZAP, ARVCF, p0071, DP1 and DP2, suggesting that these molecules are El Bagre-EPF antigens. Further, all of these antigens represent components of cell junctions, indicating that the immune response is directed, at least partially, against cell junctions. The immune response in patients affected by El Bagre-EPF is polyclonal, and it includes B and T lymphocytes, mast cells, IgG, IgA, IgM, IgD, IgE, fibrinogen, albumin, complement/C1q, C3c and C4.
Background El Bagre endemic pemphigus foliaceus (El Bagre‐EPF) is a new variant of endemic pemphigus foliaceus present in the El Bagre area of Colombia, South America. Here, we investigate the presence of complement/C5‐b9 in lesional skin of patients and matched controls from the endemic area. We also aim to compare the patient's autoantibody levels using indirect immunofluorescent titers (IIF) and correlate with the lesional presence of complement/C5b‐9. Methods A case‐control study was carried out by testing for the presence of complement/C5b‐9 in lesional skin in 43 patients affected by El Bagre‐EPF, as well as 43 matched, healthy controls from the endemic area. Skin biopsies were obtained and evaluated via hematoxylin and eosin staining, and immunohistochemistry. Results The presence of complement/C5b‐9 was observed in all cases of the patients affected by El Bagre‐EPF and was not observed in the controls from the endemic area (P < 0.001). The patients' autoantibody titers utilizing IIF for IgG and IgM showed correlation between higher autoantibody titers and stronger intensity of staining with complement/C5‐b9 staining (P < 0.001). Conclusion Patients affected by El Bagre‐EPF have lesional deposition of complement/C5b, which correlates with disease severity and previously established serologies.
Background: A new variant of endemic pemphigus foliaceus in El Bagre (El Bagre-EPF), Colombia, South America, shares features with Senear-Usher syndrome and occurs in an endemic fashion. Patients affected by El Bagre-EPF have heterogeneous antigenic reactivity not only to the skin but to other organs, including the heart. Here we test for autoantibodies to the areae compositaeof the heart (structure consisting of typical desmosomal amalgamated fascia adherensmolecules)and evaluate any possible clinical correlation. Methods: A case-control study comparing 45 patients and 45 controls from the endemic area, matched by demographics including age, gender, weight, work activities, and comorbidities, was performed. Direct and indirect immunofluorescence, immunohistochemistry, confocal microscopic studies, and echocardiogram studies were completed. Results: The main clinical abnormally seen in the El Bagre-EPF patients was left ventricular hypertrophy in 15/45 patients, compared with no such findings in the control population (P < 0.1). Seventy percent of El Bagre-EPF patients and none of the controls displayed polyclonal autoreactivity using different immunoglobulins and complement to the areae compositae of the heart using different methods and antibodies (P < 0.1). Conclusions: Patients affected by El Bagre-EPF demonstrated autoantibodies to the areae compositae of the heart. This finding was associated with left ventricular hypertrophic cardiomyopathy.The areae compositaemay play a role incell junction tension and the El Bagre-EPF patients’ autoantibodies possibly disrupting these junctions and thereby contributing to the left ventricular hypertrophy.
Background: We have characterized a new variant of endemic pemphigus foliaceus in El Bagre (El Bagre-EPF) (AKA pemphigus Abreu-Manu) and surrounding municipalities. Herein, we describe nail alterations in several patients affected by this disease. In the pre-steroid era, patients with endemic pemphigus foliaceus (EPF), especially in Brazil where the disease is known as fogo selvagem (FS), were described to have some nails changes including the Viera's sign (yellowish of the nail). In this study, we have attempted to describe a range of nail alterations in patients affected by El Bagre-EPF. Methods: A case-controlled study was conducted where 40 cases and 40 controls were evaluated for nail changes. A clinical exam was performed in the cases and in a series of control patients from the same geographic region who were matched by age, gender and work activities. Gram stains and cells cultures for fungus and bacteria were done on affected nails. Results: In 25 chronic patients (affected for more than two decades) presented with toenail alterations. These changes included change of color nail (yellowish) (Viera sign), atrophy, dystrophy, chronic paronychia, onycholysis, nail bed erosion, subungual hyperkeratosis and trachyonychia. All of these findings were overrepresented in patients compared with control population (p < 0.05). Cell cultures and gram stains were negative in all study participants. Discussion: Chronic patients have nail damage, maybe due to the presence of chronic inflammatory process affecting the nail bed cell, matrix, and/or the nail fold cells junctions.
Background A new variant of endemic pemphigus foliaceus (EPF) has been documented, El Bagre-EPF. We aimed to study antinuclear antibodies (ANAs) in these patients. Methods We performed a case-control study, testing 57 patients affected by this disease and 57 controls from the endemic area matched by work activity and demographics. The participants were evaluated clinically as well as by detection of ANAs utilizing HEp-2 cells. We utilized Triton-induced partial permeabilization of the cell membranes, allowing for the visualization of intracellular and intranuclear antigens. We also immunoadsorbed the ANAs using synthetic peptides to elucidate the nature of the ANA. Results We detected the presence of a new pattern of ANAs. The new pattern of ANAs was seen in 24% of the El Bagre-EPF patients, compared to our controls (P < 0.001). The new ANA pattern consisted of a thin nuclear and nucleolar rim, finely speckled nucleolar, nuclear membrane pores stains, and a positive intranuclear stain directed against small nuclear components, as well as cytoplasmic deposits of autoantibodies were also observed. The new ANAs pattern perfectly colocalized with commercial antibodies to miocardium-enriched zonula occlusans-1 associated protein (MIZAP), armadillo repeat gene deleted in velo-cardio-facial syndrome (ARVCF), p0071 and desmoplakins I–II (all from Progen Biotechnik). Additionally in 14% of patients with El Bagre-EPF forme fruste and hyperpigmented clinical presentations, a classic homogeneous ANA pattern was observed with autoantibodies specific for Ro, La, Sm, and double-stranded DNA antigens. Immunoadsorption with peptide-based sequences from MIZAP, ARVCF, p0071 and desmoplakins I–II removed the new ANA pattern. Conclusions We describe a new pattern of ANAs in El Bagre-EPF, colocalizing with autoantibodies directed against MIZAP, ARVCF, p0071, and desmoplakins I–II.
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