Patients with a new variant of endemic pemphigus foliaceus have autoantibodies against arrector pili muscle, colocalizing with MYZAP, p0071, desmoplakins 1 and 2 and ARVCF

Abstract

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.

Untitled

“…El Bagre-EPF patients demonstrate complex clinical, epidemiological and immunopathologic features, differing from Fogo selvagem and the Tunisian EPF [1][2][3][4][5][6][7][8]. El Bagre-EPF patients have polyclonal autoantibodies directed not only to epidermal cell junctions, but also to the cell junctions in the skin appendices, their neurovascular bundles and to mesenchymal-endothelial cell junctions [1][2][3][4][5][6][7][8]. The main laboratory findings found in El Bagre-EPF patients include autoantibodies to multiple cell junctions in the skin, and in one third of the patients against cells junctions in multiple organs [1][2][3][4][5][6][7][8].…”

“…Pemphigus foliaceus (PF) is an autoimmune skin disorder characterized by the loss of intercellular adhesion of keratinocytes in the upper parts of the epidermis (acantholysis), resulting in the formation of superficial blisters [1,2]. It has an endemic forms, and previously, we described a new variant of endemic pemphigus foliaceus (EPF) in El Bagre, Colombia (El Bagre-EPF), (AKA pemphigus Abreu-Manu).…”

“…It has an endemic forms, and previously, we described a new variant of endemic pemphigus foliaceus (EPF) in El Bagre, Colombia (El Bagre-EPF), (AKA pemphigus Abreu-Manu). It is an autoimmune disease presenting in a circumscribed geographic area with genetic and environmental factors influencing its pathogenesis [1]. This new variant, El Bagre-EPF, occurs in a gold-mining region; it predominantly affects males between 30 and 60 years of age, as well as a few post-menopausal females [1][2][3][4][5][6][7][8].…”

“…It is an autoimmune disease presenting in a circumscribed geographic area with genetic and environmental factors influencing its pathogenesis [1]. This new variant, El Bagre-EPF, occurs in a gold-mining region; it predominantly affects males between 30 and 60 years of age, as well as a few post-menopausal females [1][2][3][4][5][6][7][8]. El Bagre-EPF patients demonstrate complex clinical, epidemiological and immunopathologic features, differing from Fogo selvagem and the Tunisian EPF [1][2][3][4][5][6][7][8].…”

“…This new variant, El Bagre-EPF, occurs in a gold-mining region; it predominantly affects males between 30 and 60 years of age, as well as a few post-menopausal females [1][2][3][4][5][6][7][8]. El Bagre-EPF patients demonstrate complex clinical, epidemiological and immunopathologic features, differing from Fogo selvagem and the Tunisian EPF [1][2][3][4][5][6][7][8]. El Bagre-EPF patients have polyclonal autoantibodies directed not only to epidermal cell junctions, but also to the cell junctions in the skin appendices, their neurovascular bundles and to mesenchymal-endothelial cell junctions [1][2][3][4][5][6][7][8].…”