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):913-5. 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

Arch Dermatol 2007 Jul;143(7):895-9

Department of Dermatology, Universidade Federal de Brasilia, Brasilia, Distrito Federal.

Background: Investigators from Brasilia, Brazil, observed several patients with a mucocutaneous disease that resembles pemphigus vulgaris clinically and histologically but with epidemiological features of fogo selvagem. Our objective was to characterize antidesmoglein 3 and antidesmoglein 1 autoantibody profiles in these unique patients who reside in Goiânia and Brasilia, Brazil, known endemic regions of fogo selvagem.

Observations: We performed serological evaluation of 8 patients with a mucocutaneous disease clinically and histologically consistent with pemphigus vulgaris, as well as 27 healthy relatives of patients with fogo selvagem who reside in these endemic areas. Read More

Braz J Med Biol Res 2006 Sep;39(9):1227-32

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

Endemic pemphigus foliaceus (EPF) is an autoimmune bullous skin disease characterized by acantholysis and antibodies against a desmosomal protein, desmoglein 1. Genetic and environmental factors contribute to development of this multifactorial disease. HLA class II and some cytokine gene polymorphisms are the only genetic markers thus far known to be associated with susceptibility to or protection from EPF. Read More

J Invest Dermatol 2006 Sep;126(9):1931-2

Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.

The endemic form of pemphigus foliaceus, fogo selvagem, is caused by IgG autoantibodies directed against desmoglein 1 (Dsg1). Hilario-Vargas and his colleagues describe a high prevalence of IgG autoantibodies against Dsg3, the target antigen of pemphigus vulgaris, in a Brazilian population where fogo selvagem is endemic, although those patients do not develop any apparent clinical phenotype of pemphigus vulgaris. Read More

Int J Dermatol 2006 Aug;45(8):914-8

Department of Dermatology, Faculty of Medicine de Jundiaí, São Paulo, Brazil.

Background: Since 1967 dermatology has used the classic technique of indirect immunofluorescence (IFI) for the detection of autoantibodies against antigens of the skin in diseased people with endemic pemphigus foliaceus. Thirty years later enzyme-linked immunosorbent assays--ELISA (rDsg1 and rDsg3) appeared as a viable option. A group of highly recognized researchers have concluded that ELISA is a simple, sensitive and highly specific method, allowing for diagnostic differentiation between pemphigus vulgaris (PV) and endemic pemphigus foliaceus (EPF). Read More

Br J Dermatol 2006 Aug;155(2):446-50

Department of Dermatology, Federal University of Rio de Janeiro School of Medicine, Rio de Janeiro, Brazil.

Background: Fogo selvagem (FS) has been described in several regions of Brazil, including the Western regions of the state of Parana. In 1990, Empinotti et al. reported case studies of 213 patients with FS that were collected from 1976 to 1988. Read More

J Invest Dermatol 2006 Sep 8;126(9):2044-8. Epub 2006 Jun 8.

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

Fogo selvagem (FS), the endemic form of pemphigus foliaceus (PF), is an autoimmune blistering disease characterized by autoantibodies against desmoglein 1. The Terena reservation of Limao Verde in Mato Grosso do Sul, Brazil, is a previously identified focus of disease. Autoantibodies against desmoglein 3 (Dsg3) have also been detected in sera from patients with FS. Read More

Clin Dermatol 2006 May-Jun;24(3):158-67

Department of Dermatology, Leiden University Medical Centre, The Netherlands.

Cutaneous allergic reactions are relatively common in tropical countries, with some caused by the tropical environment and others by changes in the traditional living conditions combined with poverty. HIV infection is a major contributory factor. A large number of reactions are eczematous. Read More

Int J Dermatol 2006 May;45(5):538-42

Clinical Research Institute, Universidad Nacional Mayor de San Marcos, Lima, Peru.

Background: Endemic pemphigus foliaceus or fogo selvagem is an autoimmune skin disease characterized by the presence of subcorneal superficial blisters and antibodies of the immunoglobulin G4 (IgG4) class specific for the desmosomal glycoprotein, desmoglein 1. In Peru, no studies have been published on the seroprevalence of antibodies against desmoglein 1 in healthy subjects from endemic foci.

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

Int J Immunogenet 2006 Apr;33(2):141-4

Human Molecular Genetics Laboratory, Department of Genetics, Federal University of Paraná, 81531-990 Curitiba, Brazil.

Pemphigus foliaceus (PF) is an autoimmune bullous epidermal disease, characterized by autoantibodies specific to the desmosomal protein desmoglein 1 (dsg1) and by acantholysis, the rupture of the cellular junctions among keratinocytes. Known also as fogo selvagem (wild fire) in Brazil, the disease has distinct epidemiological characteristics, being endemic in certain regions of South America. It is a multifactorial (complex) disease, with oligo- or polygenic disease susceptibility. Read More

J Dermatol 2005 Jul;32(7):574-80

Department of Dermatology of University of Sao Paulo, Brazil.

Pemphigus foliaceus is an autoimmune cutaneous disease with subcorneal acantholysis and pathogenic IgG4 autoantibodies directed against desmoglein 1. We present our experience with mycophenolate mofetil (MMF) in the treatment of one case of endemic pemphigus foliaceus (fogo selvagem) and two cases of the classic form. All patients had severe, refractory disease and developed marked adverse effects due to long-term corticosteroid therapy. Read More

J Autoimmun 2005 Sep 19;25(2):121-5. Epub 2005 Oct 19.

Human Molecular Genetics Laboratory, Department of Genetics, Federal University of Paraná, Caixa Postal 19071, 81531-990 Curitiba, Brazil.

Transmembrane proteins of the cadherin superfamily, the desmogleins and desmocollins, mediate intercellular adhesion in desmosomes. Autoantibodies to desmoglein 1 (dsg1) are a hallmark of pemphigus foliaceus (PF), a disease characterized by skin blistering resulting from keratinocyte cell detachment. The etiology and pathogenesis of this disease remain poorly understood; however, genetic susceptibility is clearly involved. Read More

Br J Dermatol 2005 Apr;152(4):650-7

Dermatology, Department of Medicine, Faculty of Medicine of Ribeirão Preto, University of São Paulo, Avenida Bandeirantes 3900, Monte Alegre, 14049-900, Ribeirão Preto, SP, Brazil.

Background: Pemphigus foliaceus (PF) is an autoimmune blistering disease of unknown aetiology, which is endemic in Brazil. Although the pathogenesis of PF is still unknown, proteins of the contact system have been implicated.

Objectives: As the components of the kinin system may interact with those of the contact system, in this study we evaluated the plasma levels of high-molecular-weight kininogen (HK) and low-molecular-weight kininogen (LK), and the activity of plasma kallikrein, tissue kallikrein and kininase II in plasma of patients with PF presenting with Nikolsky's sign. Read More

Int J Dermatol 2005 Apr;44(4):293-8

Dermatology Department, University Hospital of Brasília, Brasília, Brazil.

Background: Endemic pemphigus foliaceus (EPF) or fogo selvagem (FS) is an endemic autoimmune disease, characterized by flaccid bullae induced by IgG(4) subclass antibodies. The authors demonstrate the epidemiological and clinical status of patients who have been followed at the University Hospital of Brasilia (HUB) for more than 15 years.

Methods: One hundred and ninety-six patients with FS took part in the project. Read More

J Invest Dermatol 2004 Dec;123(6):1045-51

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

Pemphigus foliaceus (PF) and the endemic form Fogo Selvagem (FS) are mediated by pathogenic antibodies to the EC1-2 domains of desmoglein-1. There is a preclinical phase with antibodies to only EC5. Based on geographic clustering of cases, FS is thought to have an, as yet unidentified, environmental trigger. Read More

Med Sci Monit 2004 Dec;10(12):CR657-61

Departamento de Dermatologia, Hospital Padre Bento-Guarulhos, São Paulo, Brazil.

Background: Research on direct immunofluorescence in pemphigus foliaceus ("fogo selvagem") has been focused mainly on the study of perilesional and lesional skin, while little attention has been given to uninvolved skin. We analyzed the frequency of IgA, IgM, IgG and its subclasses (IgG1, IgG2, IgG3 and IgG4) and C3 complement fraction deposition in intercellular spaces (ICS) and basal membrane zone (BMZ) in uninvolved, lesional and perilesional skin from 47 fogo selvagem patients.

Material/methods: For each patient biopsies were collected from lesional, perilesional and uninvolved skin, and then analyzed by direct immunofluorescence. Read More

Rev Hosp Clin Fac Med Sao Paulo 2004 Oct 29;59(5):251-6. Epub 2004 Oct 29.

Department of Dermatology and Immunodermatopathology Laboratory, Hospital das Clínicas, Faculty of Medicine, University of São Paulo - São Paulo/SP, Brazil.

Unlabelled: Pemphigus are autoimmune intraepidermal blistering diseases in which immunoglobulin G (IgG) autoantibodies are directed against desmosomal glycoproteins. The aim of this study was to determine the IgG subclass profile of endemic pemphigus foliaceus (fogo selvagem) and pemphigus vulgaris utilizing indirect immunofluorescence.

Patients And Methods: Twenty-five patients with pemphigus vulgaris, 25 with endemic pemphigus foliaceus (fogo selvagem), and 25 healthy controls were analyzed by indirect immunofluorescence for circulating autoantibodies (total IgG and its subclasses). Read More