Pathogenesis of Endemic Pemphigus Foliaceus

Aoki, Valéria; Sousa, Joaquim X.; Díaz, Luis A.

doi:10.1016/j.det.2011.03.014

Cited by 23 publications

(27 citation statements)

References 24 publications

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“…The antigenic target is the extracellular portion of desmoglein 1, an adhesion molecule of the cadherin superfamily, which is recognized by pathogenic IgG, especially of the IgG4 isotype. 6,22 The recognition of Dsg 1 epitopes is not restricted to patients with the disease, as previously reported. 8 In this study, we detected 22% of reactivity against Dsg1 in non-FS individuals, indicating a possible environmental stimulus in the development of autoantibody formation.…”

Section: Discussionsupporting

confidence: 52%

“…A sequence of eight amino acids (LLEQRRAA) at the positions 67-74 in the third hypervariable domain of the DRB1 gene is shared by these alleles, conferring susceptibility to the disease. 21,22 In genetically predisposed individuals, there may be triggers that initiate the immune response in FS through an antigen mimicry process. 16 It is hypothesized that a break of immune tolerance follows exposure to some environmental factor(s) that include hematophagous insect bites, as reported elsewhere.…”

Section: Introductionmentioning

confidence: 99%

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Profile of Trypanosoma cruzi Reactivity in a Population at High Risk for Endemic Pemphigus Foliaceus (Fogo Selvagem)

Sousa

Díaz²,

Eaton

et al. 2012

The American Society of Tropical Medicine and Hygiene

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Abstract. Fogo Selvagem (FS) is an autoimmune bullous disease with pathogenic IgG autoantibodies recognizing desmoglein 1 (Dsg1), a desmosomal glycoprotein. In certain settlements of Brazil, a high prevalence of FS (3%) is reported, suggesting environmental factors as triggers of the autoimmune response. Healthy individuals from endemic areas recognize nonpathogenic epitopes of Dsg1, and exposure to hematophagous insects is a risk factor for FS. Fogo selvagem and Chagas disease share some geographic sites, and anti-Dsg1 has been detected in Chagas patients. Indeterminate Chagas disease was identified in a Brazilian Amerindian population of high risk for FS. In counterpart, none of the FS patients living in the same geographic region showed reactivity against Trypanosoma cruzi. The profile of antiDsg1 antibodies showed positive results in 15 of 40 FS sera and in 33 of 150 sera from healthy individuals from endemic FS sites, and no cross-reactivity between Chagas disease and FS was observed.

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Section: Discussionsupporting

confidence: 52%

Section: Introductionmentioning

confidence: 99%

Profile of Trypanosoma cruzi Reactivity in a Population at High Risk for Endemic Pemphigus Foliaceus (Fogo Selvagem)

Sousa

Díaz²,

Eaton

et al. 2012

The American Society of Tropical Medicine and Hygiene

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“…Most cases of PF are sporadic and account for 20–30% of pemphigus cases. However, endemic forms of PF (also known as fogo selvagem) occur in regions of Brazil, Columbia and Tunisa (Aoki, et al, 2011, Joly and Litrowski, 2011). Brazilian endemic pemphigus foliaceus patients in particular also generate autoantibodies to Dsc 1 and 2 (Dmochowski, et al, 1993).…”

Section: Acquired Desmosomal Diseasesmentioning

confidence: 99%

Desmosomes in acquired disease

Stahley

Kowalczyk

2015

Cell Tissue Res

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Desmosomes are cell-cell junctions that mediate adhesion and couple the intermediate filament cytoskeleton to sites of cell-cell contact. This architectural arrangement functions to integrate adhesion and cytoskeletal elements of adjacent cells. The importance of this robust adhesion system is evident in numerous human diseases, both inherited and acquired, that occur when desmosome function is compromised. This review focuses on autoimmune and infectious diseases that impair desmosome function. In addition, we discuss emerging evidence that desmosomal genes are often misregulated in cancer. The emphasis of our discussion is placed on how human diseases inform our understanding of basic desmosome biology, and in turn, how fundamental advances in the cell biology of desmosomes may lead to new treatments for acquired diseases of the desmosome.

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“…31 In the normal population, IgG4 is usually found in reduced circulating levels; it is augmented under specific conditions, such as in patients taking immunotherapy, where IgG4 is used as a predictive marker, 32 or in autoimmune diseases, 33 such as endemic pemphigus foliaceus, where IgG4 is pathogenic. 34 Additionally, we found diminished IgG1 and IgG3 antibody levels directed against SEB according to disease severity. These subclasses are prominent in the S. aureus response and are capable of fixing complement, and with disease progression, a major immunologic response compromise might occur.…”

Section: Discussionmentioning

confidence: 94%