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J Eur Acad Dermatol Venereol 2022 May 24. Epub 2022 May 24.

Research center of big data and meta-analysis, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan.

J Invest Dermatol 2021 11 13;141(11):2741-2744. Epub 2021 May 13.

Programa de Pós-Graduação em Genética, Universidade Federal do Paraná, Curitiba, Brazil. Electronic address:

J Am Acad Dermatol 2021 Jun 5;84(6):1507-1519. Epub 2021 Mar 5.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.

Intraepithelial autoimmune blistering dermatoses are a rare group of skin disorders characterized by the intraepithelial disruption of intercellular connections through the action of autoantibodies. The first article in this continuing medical education series explores the background, epidemiology, clinical features, and diagnostic criteria of each of the major intraepithelial autoimmune blistering dermatoses, including pemphigus foliaceus, pemphigus erythematosus, pemphigus herpetiformis, fogo selvagem, pemphigus vulgaris, pemphigus vegetans, drug-induced pemphigus, IgA pemphigus, IgG/IgA pemphigus, and paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome. Read More

J Autoimmun 2021 01 4;116:102561. Epub 2020 Nov 4.

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

Epitope spreading is an important mechanism for the development of autoantibodies (autoAbs) in autoimmune diseases. The study of epitope spreading in human autoimmune diseases is limited due to the major challenge of identifying the initial/primary target epitopes on autoantigens in autoimmune diseases. We have been studying the development of autoAbs in an endemic human autoimmune disease, Brazilian pemphigus foliaceus (or Fogo Selvagem (FS)). Read More

Sci Rep 2020 10 19;10(1):17664. Epub 2020 Oct 19.

Laboratory of Malaria and Vector Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health (NIH), Rockville, Maryland, USA.

Leishmaniasis encompasses a spectrum of diseases caused by a protozoan belonging to the genus Leishmania. The parasite is transmitted by the bite of sand flies, which inoculate the promastigote forms into the host's skin while acquiring a blood meal. Nyssomyia neivai is one of the main vectors of tegumentary leishmaniasis (TL) in Brazil. Read More

J Invest Dermatol 2020 12 29;140(12):2332-2342.e10. Epub 2020 Apr 29.

Vector Molecular Biology Section, Laboratory of Malaria and Vector Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Rockville, Maryland, USA.

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 nonpathogenic anti-DSG1 antibodies. Recurring bites from Lutzomyia longipalpis, the sand fly vector of leishmaniasis, immunize individuals with L. 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

Br J Dermatol 2019 08 21;181(2):324-331. Epub 2019 Apr 21.

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

Background: Pemphigus foliaceus (PF) is an epidermal autoimmune disease, characterized by the presence of autoantibodies against the desmosomal protein desmoglein 1. Genetic and environmental factors contribute to PF, a complex disease that is endemic in Brazil and Colombia and neighbouring countries, and in Tunisia. Long noncoding RNAs (lncRNAs) may participate in gene regulation by interacting with DNA, proteins and other RNAs. 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 03;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.