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J Invest Dermatol 2020 Apr 29. Epub 2020 Apr 29.

Vector Molecular Biology Section, LMVR, NIAID, NIH, Rockville, MD, 20852.

Fogo Selvagem (FS) is a blistering skin disease caused by pathogenic IgG4 autoantibodies to desmoglein-1 (Dsg1). Preclinical FS and Leishmaniasis are endemic to certain regions of Brazil and exhibit non-pathogenic anti-Dsg1 antibodies. Recurring bites from Lutzomyia longipalpis (LP), the sand-fly vector of Leishmaniasis, immunize individuals with LP salivary antigens LJM17/LJM11. Read More

Front Immunol 2019 22;10:2585. Epub 2019 Nov 22.

Laboratory of Human Molecular Genetics, Department of Genetics, Federal University of Paraná, Curitiba, Brazil.

Pemphigus foliaceus is an autoimmune disease that is sporadic around the world but endemic in Brazil, where it is known as fogo selvagem (FS). Characterized by autoantibodies against the desmosomal cadherin desmoglein 1, FS causes painful erosions, and crusts that may be widespread. The recognition of antigens, including exposed sugar moieties, activates the complement system. Read More

J Cutan Pathol 2019 Dec 9;46(12):925-929. Epub 2019 Sep 9.

Georgia Dermatopathology Associates, Atlanta, Georgia.

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. Read More

J Exerc Rehabil 2019 Jun 30;15(3):460-467. Epub 2019 Jun 30.

Federal University of Triângulo Mineiro, Uberaba, Brazil.

Fogo Selvagem (FS) is a rare autoimmune disease characterized by acantholysis and inflammation of the epidermis. It was evidenced in this disease the increase of proinflammatory cytokines levels which can be influenced by physical activities. Kinesiotherapy, as physiotherapeutic interventions, was associated improvement levels of the quality of live, mainly the pain. Read More

Br J Dermatol 2019 08 17;181(2):241-242. Epub 2019 Jul 17.

Department of Dermatology, University of Illinois at Chicago, 808 S Wood St, RM377, Chicago, IL, 60612, U.S.A.

Int J Immunogenet 2019 Jun 18;46(3):139-145. Epub 2019 Mar 18.

Laboratório de Genética Molecular Humana, Departamento de Genética, Universidade Federal do Paraná, Curitiba, Brazil.

Pemphigus foliaceus (PF) is an autoimmune skin disease characterized by autoantibodies directed mainly against desmoglein-1. The purpose of this study was to determine whether differential susceptibility to endemic PF in Brazil (fogo selvagem) is associated with polymorphisms at the cytogenetic location 1p13.2. Read More

An Bras Dermatol 2018 Sep-Oct;93(5):638-650

Dermatology Service, Hospital Maria Aparecida Pedrossian, Universidade Federal de Mato Grosso do Sul, Campo Grande (MS), Brazil.

Fogo selvagem or endemic pemphigus foliaceus is an autoimmune acantholytic anti-cadherin bullous disease that primarily affects seborrheic areas, which might disseminate. Brazil has the world's largest number of patients, mainly in the Central-West region, but the disease has also been reported in other South American countries. It affects young people and adults who have been exposed to rural areas, with occurrence of familial cases. Read More

An Bras Dermatol 2018 Mar;93(2):212-215

Department of Dermatology, Oregon Health & Science University, Oregon, USA.

Background: Previous studies have shown oxidative stress in pemphigus vulgaris and pemphigus foliaceus, nevertheless, it remains unknown whether a similar response is characteristic of endemic pemphigus foliaceus in Peru.

Objectives: To determine the oxidative stress response in endemic pemphigus foliaceus patients and subjects with positive for anti-desmoglein1 antibodies (anti-dsg1) from endemic areas of Peru.

Subjects And Methods: This is a cross-sectional study. Read More

J Eur Acad Dermatol Venereol 2018 Nov 6;32(11):1954-1958. Epub 2018 Apr 6.

Department of Dermatology, University of São Paulo Medical School, São Paulo, São Paulo, Brazil.

Background: Erythroderma is a severe manifestation of pemphigus foliaceus (PF), a blistering disease mediated by IgG autoantibodies against desmoglein 1. Increasing evidence supports the contribution of angiogenic mediators in the pathogenesis of erythroderma.

Objective: To evaluate the in situ expression of vascular endothelial growth factor (VEGF) and endoglin in patients with PF with erythroderma. Read More

Heart Rhythm 2018 05 31;15(5):732-733. Epub 2018 Jan 31.

Heart Rhythm Division, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.

J Autoimmun 2018 05 4;89:171-185. Epub 2018 Jan 4.

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA. Electronic address:

Fogo Selvagem (FS), the endemic form of pemphigus foliaceus, is mediated by pathogenic IgG4 autoantibodies against the amino-terminal extracellular cadherin domain of the desmosomal cadherin desmoglein 1 (Dsg1). Here we define the detailed epitopes of these pathogenic antibodies. Proteolytic footprinting showed that IgG4 from 95% of FS donor sera (19/20) recognized a 16-residue peptide (ALNSMGQDLERPLELR) from the EC1 domain of Dsg1 that overlaps the binding site for an adhesive-partner desmosomal cadherin molecule. Read More

Heart Rhythm 2018 05 24;15(5):725-731. Epub 2017 Dec 24.

Department of Cardiology, Hospital General de Medellin, and Clinica CES Medellin, Antioquia, Colombia.

Background: We previously showed that one-third of patients affected by endemic pemphigus foliaceus in El Bagre, Colombia (El Bagre-EPF), display autoreactivity to the heart.

Objective: The purpose of this study was to investigate rhythm disturbances with the presence of autoantibodies and correlate them with ECG changes in these patients.

Methods: We performed a study comparing 30 patients and 30 controls from the endemic area, matched by demographics, including age, sex, weight, work activities, and comorbidities. Read More

Clin Exp Dermatol 2017 Dec 15;42(8):874-880. Epub 2017 Oct 15.

Georgia Dermatopathology Associates, Atlanta, GA, USA.

Background: We identified a new variant of endemic pemphigus foliaceus in El Bagre, Colombia, South America, which we term El Bagre-EPF, and observed reactivity to arrector pili muscle (APM), thus we tested for autoimmunity to APM.

Methods: We took skin biopsies from 30 patients with El Bagre-EPF and 30 healthy controls (HCs) matched by age, sex and occupation, who were all from the endemic area, and tested these using direct immunofluorescence (DIF), confocal microscopy, immunohistochemistry and immunoblotting (IB).

Results: Of the 30 patients with El Bagre-EPF, 27 had autoantibodies to APM that colocalized with commercial antibodies to myocardium-enriched zonula occludens-1-associated protein (MYZAP), desmoplakin (DP)1 and DP2, plakophilin 4, and Armadillo repeat gene deleted in velo-cardio-facial syndrome (ARVCF) (P < 0. Read More

Immunohorizons 2017 Aug;1(6):71-80

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599.

We have shown that although the IgG response in fogo selvagem (FS) is mainly restricted to desmoglein (Dsg) 1, other keratinocyte cadherins are also targeted by FS patients and healthy control subjects living in the endemic region of Limão Verde, Brazil (endemic controls). Evaluating nonpathogenic IgG1 and pathogenic IgG4 subclass responses to desmosomal proteins may reveal important differences between pathogenic and nonpathogenic responses, and how these differences relate to the pathogenic IgG4 response and resultant FS. In this study, we tested by ELISA >100 sera from each FS patient, endemic control, and nonendemic control for IgG1 and IgG4 autoantibodies to keratinocyte cadherins besides Dsg1. Read More

Front Immunol 2017 14;8:978. Epub 2017 Aug 14.

Laboratory of Immunology, Institute of Natural and Biological Sciences, Federal University of Triângulo Mineiro, Uberaba, Brazil.

Introduction: The endemic form (fogo selvagem-FS) of pemphigus foliaceus is an autoimmune disease characterized by the presence of IgG autoantibodies against desmoglein-1. Despite the array of findings, the role of chemokines and cytokines that dictate the immune response and disease outcome is still poorly investigated.

Materials And Methods: Serum from 64 patients diagnosed with FS was used to draw and establish the levels of these molecules on this disease and establish the levels of these molecules with the severity of FS, and influence of treatment. Read More

Immunol Lett 2017 04 16;184:105-111. Epub 2017 Feb 16.

Immunology Department, Habib Bourguiba Hospital, University of Sfax, Sfax, Tunisia. Electronic address:

Objective: Forkhead box P3 (FOXP3) is an essential and crucial transcription factor of regulatory T-cells. Genetic polymorphisms in the promoter region of FOXP3 gene may alter the gene expression level and, therefore, contribute to several autoimmune diseases susceptibility. We aimed to investigate the possible role of genetic variants of four SNPs (rs3761547, rs3761548, rs3761549 and rs2294021) and a (GT) microsatellite located in FOXP3 gene in the susceptibility to Tunisian Pemphigus Foliaceus (PF). Read More

Clin Lab 2016 Jul;62(7):1209-1216

Background: Pemphigus is a group of autoimmune blistering diseases of which the major forms are pemphigus foliaceus (PF) and vulgaris (PV). In Brazil, PF occurs in an endemic form also known as fogo selvagem. The main autoantibody in PF is against desmoglein 1 (DSG1), while in PV the main antibody is anti-desmoglein 3 (DSG3), but often anti-DSG1 is also present. Read More

J Eur Acad Dermatol Venereol 2016 Oct 15;30(10):e57-e59. Epub 2015 Sep 15.

Department of Dermatology, University of São Paulo Medical School, São Paulo, Brazil.

Autoimmun Rev 2016 Sep 8;15(9):923-30. Epub 2016 Jul 8.

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

Pemphigus represents a group of organ specific autoimmune blistering disorders of the skin mediated by pathogenic autoantibodies with well-defined antigenic targets. While most of these diseases are sporadic, endemic forms of disease do exist. The endemic form of pemphigus foliaceus (also known as fogo selvagem, FS) exhibits epidemiological features that suggest exposure to hematophagous insect bites are a possible precipitating factor of this autoimmune disease, and provides a unique opportunity to study how environmental factors contribute to autoimmune disease development. Read More

J Immunol 2016 Mar 29;196(5):2041-50. Epub 2016 Jan 29.

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599;

The etiology of human autoimmune diseases in general remains largely unknown, although the genetic and environmental interplay may be relevant. This applies to the autoimmune diseases of the skin such as the pemphigus phenotypes and others. In this group, there is an endemic form of pemphigus foliaceus (also known as fogo selvagem [FS]) in which the pathogenic IgG4 autoantibody response to the self-antigen desmoglein 1 (Dsg1) cross-reacts with the LJM11 sand fly salivary gland Ag. Read More

Semin Immunopathol 2016 Jan 23;38(1):57-74. Epub 2015 Nov 23.

Vetsuisse Faculty, DermFocus, Bern, Switzerland.

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are two severe autoimmune bullous diseases of the mucosae and/or skin associated with autoantibodies directed against desmoglein (Dsg) 3 and/or Dsg1. These two desmosomal cadherins, typifying stratified epithelia, are components of cell adhesion complexes called desmosomes and represent extra-desmosomal adhesion receptors. We herein review the advances in our understanding of the immune response underlying pemphigus, including human leucocyte antigen (HLA) class II-associated genetic susceptibility, characteristics of pathogenic anti-Dsg antibodies, antigenic mapping studies as well as findings about Dsg-specific B and T cells. Read More

J Dermatol 2015 Jan;42(1):18-26

Department of Dermatology, University of São Paulo Medical School, São Paulo, Brazil.

Pemphigus are organ-specific autoimmune diseases, where autoantibodies (mainly immunoglobulin [Ig]G) directed against epidermal targets (glycoproteins of the desmosomal core) are detected. Endemic pemphigus foliaceus or fogo selvagem (FS) is one of the variants of pemphigus foliaceus pemphigus foliaceus that shares the same clinical and immunopathological features of the classic non-endemic pemphigus foliaceus form, including pathogenic IgG (mainly IgG4) autoantibodies directed against the ectodomain of desmoglein 1 (Dsg1), that lead to acantholysis. Pathogenesis of FS is complex, involving genetic, environmental and immunological factors. Read More

J Clin Exp Dermatol Res 2014 Feb;5(2)

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.

Objectives: Fogo Selvagem (FS) in Limao Verde (LV), Brazil shows clinical and histological features of pemphigus foliaceus (PF) and shares pathogenic IgG4 anti-desmoglein 1 (Dsg1) autoantibodies. Previously, our group reported that mothers with active FS deliver babies with normal skin and low/negative titers of IgG4 autoantibodies by indirect immunofluorescence. It was postulated that maternal pathogenic IgG4 autoantibodies do not cross the placenta due to differential receptor mediated transplacental passage of IgG subclasses. Read More

J Invest Dermatol 2015 Mar 6;135(3):913-915. Epub 2014 Oct 6.

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.

Br J Dermatol 2013 Oct;169(4):812-8

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC, 27599, U.S.A; Duke University School of Medicine, Durham, NC, 27710, U.S.A.

Background: Pemphigus vulgaris (PV) is an autoimmune skin blistering disease. The main targets of autoantibodies are the desmosomal proteins desmoglein (Dsg)3 and Dsg1. Anti-E-cadherin antibody is the second most frequent antibody found in pemphigus foliaceus (fogo selvagem), but the frequency in PV is unknown. Read More

J Invest Dermatol 2012 Nov;132(11):2499-502

Laboratory of Molecular and Cell Biology, Istituto Dermopatico dell'Immacolata (IDI-IRCCS), Rome, Italy.

Fogo selvagem is an endemic form of pemphigus foliaceus (EPF) found in Brazil. Environmental and genetic factors are thought to contribute to the disease, which is associated with pathogenic IgG4 autoantibodies against the desmosomal cadherin desmoglein 1. In this issue, as an additional framework to understand autoimmune mechanisms in EPF, Flores et al. Read More

Am J Trop Med Hyg 2012 Oct 23;87(4):675-80. Epub 2012 Jul 23.

Department of Dermatology, University of São Paulo Medical School, São Paulo, Brazil.

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. Read More

J Invest Dermatol 2012 Nov 19;132(11):2573-80. Epub 2012 Jul 19.

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.

It is well established that autoantibodies against desmoglein 3 and desmoglein 1 (Dsg1) are relevant in the pathogenesis of pemphigus vulgaris and pemphigus foliaceus, including its endemic form fogo selvagem (FS). Isolated reports have shown that in certain patients with these diseases, autoantibodies against other desmosomal cadherins and E-cadherin may also be present. The goal of this investigation was to determine whether FS patients and normal individuals living in endemic areas possess autoantibodies against other desmosomal cadherins and E-cadherin. Read More

J Immunol 2012 Aug 13;189(4):1535-9. Epub 2012 Jul 13.

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

The environmental factors that contribute to the development of autoimmune diseases are largely unknown. Endemic pemphigus foliaceus in humans, known as Fogo Selvagem (FS) in Brazil, is mediated by pathogenic IgG4 autoantibodies against desmoglein 1 (Dsg1). Clusters of FS overlap with those of leishmaniasis, a disease transmitted by sand fly (Lutzomyia longipalpis) bites. Read More

Am J Trop Med Hyg 2012 Jun;86(6):1005-14

Laboratory of Malaria and Vector Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Rockville, MD 20892, USA.

Triatoma matogrossensis is a Hemiptera that belongs to the oliveirai complex, a vector of Chagas' disease that feeds on vertebrate blood in all life stages. Hematophagous insects' salivary glands (SGs) produce potent pharmacologic compounds that counteract host hemostasis, including anticlotting, antiplatelet, and vasodilatory molecules. Exposure to T. Read More

Med Hypotheses 2012 Jan 13;78(1):60-6. Epub 2011 Oct 13.

In endemic pemphigus foliaceus (EPF) or "fogo selvagem" the epidemiological evidence shows that all the described outbreaks occur on the banks of rivers where there is mercury contamination from alluvium gold mining and deforestation. Pathophysiological evidence shows a similarity to pemphigus induced by sulphydryl (SH-) drugs that act by denaturing cadherins at the desmosomal level, which are the pemphigus antigens. The sulfhydryl radical (SH-) call also thiol or mercaptans from the SH-drugs, act at the level of SH-groups of cystein as would the methyl mercury from the contaminated animals and fish in the diet of humans from endemic areas of pemphigus foliaceus. Read More

Genet Mol Biol 2010 Jul 1;33(3):442-4. Epub 2010 Sep 1.

Laboratório de Genética Molecular Humana, Departamento de Genética, Universidade Federal do Paraná, Curitiba, PR Brazil.

Pemphigus foliaceus is an organ-specific autoimmune disease characterized by autoantibodies against the extracellular region of desmoglein 1, a protein that mediates intercellular adhesion in desmosomes. Cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) is a key negative regulator of the T cell immune response, playing an important role in T cell homeostasis and maintenance of peripheral tolerance. Polymorphisms in the CTLA4 gene have been associated with autoimmune diseases and the functional CT60 single nucleotide polymorphism (rs3087243, also named 6230G > A) has been proposed to be a casual variant in several of these diseases. Read More

Dermatol Clin 2011 Jul;29(3):413-8, viii

Department of Dermatology, University of São Paulo Medical School, Avenida Doutor Eneas de Carvalho Aguiar, 255 Sala 3016 ICHC, São Paulo CEP 05403-002, Brazil.

Pemphigus refers to a group of human autoimmune blistering diseases involving skin and/or mucous membranes. Endemic pemphigus foliaceus (EPF), or fogo selvagem is an organ-specific autoimmune blistering disease, first reported in the beginning of the 20th century in rural areas of Brazil. The disease follows the course of streams and creeks, and vanishes after urbanization of the endemic areas. Read More

Eur J Dermatol 2011 May-Jun;21(3):371-5

Georgia Dermatopathology Associates, Atlanta, GA, USA.

Senear Usher syndrome is a variant of pemphigus foliaceus, confined to seborrheic sites and considered to be a clinical overlap syndrome, with features of both pemphigus foliaceus and lupus erythematosus. We recently described autoantibodies to skin eyelid meibomian glands in patients with a new variant of endemic pemphigus foliaceus (El Bagre EPF) in South America. We tested for El Bagre EPF patient sera autoreactivity to pilosebaceous units utilizing direct and indirect immunofluorescence, confocal microscopy, immunohistochemistry and immunoelectron microscopy. Read More

J Clin Immunol 2011 Jun 6;31(3):356-68. Epub 2011 Jan 6.

Georgia Dermatopathology Associates, 1534 North Decatur Rd. NE, Suite 206, Atlanta, GA 30307-1000, USA.

Background: Endemic pemphigus foliaceus (EPF), is also known as "fogo selvagem" or "wild fire," reflecting the intense burning sensation of the skin reported by patients with this disease. Based on this finding, we tested for neural autoreactivity in patients affected by a new variant of EPF (El Bagre-EPF).

Methods: We tested 20 El Bagre-EPF patients, 20 normal controls from the endemic area, and 20 age- and sex-matched normal controls from outside the endemic area. Read More

J Invest Dermatol 2011 Apr 30;131(4):985-7. Epub 2010 Dec 30.

Am J Trop Med Hyg 2010 Jun;82(6):1060-75

Laboratory of Malaria and Vector Research, National Institute of Allergy and Infectious Diseases, Rockville, MA, USA.

Pemphigus foliaceus is a life threatening skin disease that is associated with autoimmunity to desmoglein, a skin protein involved in the adhesion of keratinocytes. This disease is endemic in certain areas of South America, suggesting the mediation of environmental factors triggering autoimmunity. Among the possible environmental factors, exposure to bites of black flies, in particular Simulium nigrimanum has been suggested. Read More

N Am J Med Sci 2010 Mar;2(3):114-25

Georgia Dermatopathology Associates, Atlanta, Georgia, USA.

Background: Endemic pemphigus foliaceus (EPF) is an autoimmune disease, classically occurring in a restricted geographic area. Foci of EPF have been described in several Central and South American countries, often affecting young people and Amerindians, with some female predilection. Although most American EPF cases have been documented in Brazil, cases have been reported in Peru, Paraguay, El Salvador and Venezuela. Read More

N Am J Med Sci 2010 Feb;2(2):51-9

Georgia Dermatopathology Associates, Atlanta, Georgia, USA.

Background: Endemic pemphigus foliaceus (EPF) is the only known autoimmune disease presenting in circumscribed geographic areas.

Aim: We aim to provide information concerning the natural course of EPF, including systemic compromise in the presteroid era, which has been largely unavailable in the current medical literature. MATERIAL #ENTITYSTARTX00026;

Methods: By a retrospective review of the literature we aim to compile and compare the focus of EPF and the current knowledge about them. Read More

Actas Dermosifiliogr 2009 Dec;100 Suppl 2:40-8

Department of Dermatology, University of North Carolina School of Medicine, Chapel Hill, North Carolina, United States of America.

Endemic pemphigus foliaceus, known as Fogo Selvagem (FS) in Brazil, is a disease characterized by subcorneal blisters and IgG4 anti-dermoglein 1 (Dsg1) autoantibodies. Epidemiological studies of FS strongly an environmental etiology. A 15-year surveillance of the Limao Verde Amerindian reservation in Brazil has uncovered information on the transition of the autoimmune response from the pre-clinical stage to disease state. Read More

Autoimmun Rev 2010 Mar 3;9(5):A379-82. Epub 2009 Nov 3.

Paul Sabatier University and Dermatology Department of University Hospital Toulouse, France.

Sporadic and endemic forms of pemphigus foliaceus exist. Incidences of the various forms of pemphigus vary from country to country: pemphigus vulgaris is most common in Europe and the USA, whereas pemphigus foliaceus is more prevalent in Northern Africa, Turkey and Southern America. Endemic pemphigus foliaceus (also known as fogo selvagem and Brazilian pemphigus foliaceus) is a variant of the disease that is frequently diagnosed in some areas of Brazil and other underdeveloped areas of the world, including Tunisia and Colombia. Read More

Eur J Dermatol 2010 Jan-Feb;20(1):74-81. Epub 2009 Nov 4.

Georgia Dermatopathology Associates, Atlanta, GA 30307-1000, USA.

We previously described a new variant of endemic pemphigus foliaceus in El Bagre, Colombia, South America (El Bagre-EPF). On physical examination, the palms and soles of El Bagre-EPF patients reveal an edematous texture and mild hyperkeratosis, in comparison with the non-glabrous skin of the patients where blisters, pustules or other lesions are commonly found. Based on the preceding observation, we tested the palms of 20 El Bagre-EPF cases and 20 controls from the endemic area for any pathological alterations in the samples by direct immunofluorescence (DIF). Read More

J Invest Dermatol 2009 Dec 2;129(12):2823-34. Epub 2009 Jul 2.

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599, USA.

Fogo selvagem (FS), the endemic form of pemphigus foliaceus (PF), is characterized by pathogenic anti-desmoglein 1 (DSG1) autoantibodies. To study the etiology of FS, hybridomas that secrete either IgM or IgG (predominantly IgG1 subclass) autoantibodies were generated from the B cells of eight FS patients and one individual 4 years before FS onset, and the H and L chain V genes of anti-DSG1 autoantibodies were analyzed. Multiple lines of evidence suggest that these anti-DSG1 autoantibodies are antigen selected. Read More

Genes Immun 2009 Sep 7;10(6):547-58. Epub 2009 May 7.

Laboratório de Genética Molecular Humana, Departamento de Genética, Universidade Federal do Paraná, 81531-990 Curitiba, Parana, Brazil.

Following the candidate gene approach we analyzed the CD40L, CD40, BLYS and CD19 genes that participate of B-cell co-stimulation, for association with pemphigus foliaceus (PF), an organ-specific autoimmune disease, characterized by the detachment of epidermal cells from each other (acantholysis) and presence of autoantibodies specific for desmoglein 1 (dsg1), an epidermal cell-adhesion molecule. The disease is endemic in certain regions of Brazil and also is known as fogo selvagem. Complex interactions among environmental and genetic susceptibility factors contribute to the manifestation of this multifactorial disease. Read More

Arch Pathol Lab Med 2009 Jan;133(1):97-100

Laboratory of Immunology, Universidade Federal do Triangulo Mineiro, 38025-180 Uberaba, Minas Gerais, Brazil.

Context: Endemic pemphigus foliaceus, or fogo selvagem, is a chronic autoimmune disease characterized by the formation of intraepidermal blisters that reduce adhesion between keratinocytes. Endemic pemphigus foliaceus is associated with the presence of autoantibodies and high levels of cytokines involved in the inflammatory response.

Objectives: To evaluate the expression of the proinflammatory cytokines interleukin 1, interferon gamma, and tumor necrosis factor alpha; the proapoptotic inducers Fas and inducible nitric oxide synthase; and the apoptosis inhibitor Bcl-2; and to evaluate the presence of apoptosis. Read More

J Autoimmun 2008 Dec 5;31(4):311-24. Epub 2008 Oct 5.

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.

Pemphigus encompasses a group of organ specific, antibody mediated autoimmune diseases of the skin characterized by keratinocyte detachment that leads to the development of blisters and erosions, which can become life-threatening. The pathogenic autoantibodies recognize desmogleins, which are members of the desmosomal cadherin family of cell adhesion molecules. Desmoglein 3 is targeted in pemphigus vulgaris while desmoglein 1 is targeted in pemphigus foliaceus and its endemic form, Fogo Selvagem. Read More

J Invest Dermatol 2009 Jan 14;129(1):110-8. Epub 2008 Aug 14.

Department of Biostatisitcs, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599, USA.

Fogo selvagem (FS) is mediated by pathogenic, predominantly IgG4, anti-desmoglein 1 (Dsg1) autoantibodies and is endemic in Limao Verde, Brazil. IgG and IgG subclass autoantibodies were tested in a sample of 214 FS patients and 261 healthy controls by Dsg1 ELISA. For model selection, the sample was randomly divided into training (50%), validation (25%), and test (25%) sets. Read More

Curr Dir Autoimmun 2008 ;10:182-94

Department of Dermatology, University of North Carolina-Chapel Hill School of Medicine, Chapel Hill, NC 27599, USA.

Pemphigus foliaceus (PF) and its endemic form fogo selvagem (FS) are autoimmune diseases characterized clinically by transient cutaneous superficial blisters. As opposed to pemphigus vulgaris (PV), patients lack mucosal involvement. Acantholysis in the upper epidermis is appreciated histologically. Read More

J Invest Dermatol 2008 Jul 24;128(7):1710-8. Epub 2008 Jan 24.

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599, USA.

Pemphigus foliaceus (PF) and pemphigus vulgaris (PV) are autoimmune blistering diseases characterized by autoantibodies against desmoglein (Dsg)1 and Dsg3, respectively. The role of classical cadherins as immunological targets of pemphigus autoantibodies is unknown. In this study, we tested the reactivity of sera from patients with PF, Fogo Selvagem (FS), and PV by immunoprecipitation coupled with immunoblotting (IP-IB) and ELISA techniques using a baculovirus-expressed ectodomain of E-cadherin. Read More

J Invest Dermatol 2008 Mar 25;128(3):667-75. Epub 2007 Oct 25.

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599, USA.

Fogo selvagem (FS) and pemphigus foliaceus (PF) possess pathogenic IgG anti-desmoglein 1-(Dsg1) autoantibodies. Although PF occurs sporadically, FS is endemic in Limao Verde (LV), Brazil (3.4% prevalence). Read More