Endemic Pemphigus foliaceus ("Fogo selvagem"): a series from the Northeastern region of the State of São Paulo, Brazil, 1973-1998

Chiossi, Maria Paula do Valle; Roselino, Ana Maria Ferreira

doi:10.1590/s0036-46652001000200001

Cited by 36 publications

(41 citation statements)

References 3 publications

(5 reference statements)

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“…Endemic pemphigus foliaceus (EPF) is an autoimmune blistering skin disease presenting in well-defined geographic regions [1][2][3][4][5][6][7]. The roles of IgG autoantibodies and complement in lesional EPF skin has been previously documented [1][2][3][4][5][6][7].…”

Section: Introductionmentioning

confidence: 99%

Mast cells, Mast/Stem Cell Growth Factor receptor (c-kit/cd117) and IgE may be integral to the pathogenesis of endemic pemphigus foliaceus

Velez¹,

Roselino²,

Howard³

2013

Our Dermatol Online

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Introduction: Pemphigus foliaceus (PF) is endemic in some South American countries, especially in Colombia and Brazil; in Brazil, it is also known as fogo selvagem (FS). We aimed to study the presence of mast cells and the expression of the mast/stem cell growth factor receptor (c-kit/CD117) in PF skin biopsies, as well as the role of IgE in the disease pathogenesis. Methods: Forty-four skin biopsies from patients affected by endemic PF (EPF) (30 patients from El Bagre, Colombia, and 14 from the northeastern region of São Paulo State, Brazil), 48 control biopsies from Colombian and Brazilian endemic areas, and additional control biopsies from none endemic areas in Colombia and the USA non were studied. Immunohistochemistry (IHC) was performed to evaluate skin biopsies with anti-mast cell tryptase (MCT), anti-c-kit and anti-IgE antibodies. We also searched for serum IgE in 30 EPF and 30 non-atopic controls from the El Bagre region via ELISA. In our El Bagre patients and controls, we also searched for IgE in skin samples by direct immunofluorescence. Results: In 100% of the EPF biopsies, MCT, c-kit and IgE were identified with stronger expression relative to control biopsies, especially in the inflammatory infiltrates around upper dermal blood vessels and dermal eccrine glands. IgE staining was positive along the BMZ in some EPF skin samples. The DIF results confirmed a linear deposition of IgE at the BMZ. Increased IgE serum levels were also noted in PF patients relative to controls.. Conclusions:In patients with EPF, the observed increased expression of MCT, c-kit and IgE in lesional skin, associated with higher serum IgE levels may indicate possible IgE participation in the antigenic response.

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Section: Introductionmentioning

confidence: 99%

Mast cells, Mast/Stem Cell Growth Factor receptor (c-kit/cd117) and IgE may be integral to the pathogenesis of endemic pemphigus foliaceus

Velez¹,

Roselino²,

Howard³

2013

Our Dermatol Online

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“…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. Some findings concerning the nonpathogenic response towards Dsg1 are relevant to reinforce the environmental hypothesis in FS, as follows: the lower prevalence of the IgG anti-Dsg1 response in normal subjects that live far away from the endemic sites (13%), 32 the predominance of a certain black fly species, Simulium nigrimanum, in FS regions, 24 an enhanced IgM or IgE anti-Dsg1 immune response in FS, 19,20 and finally, the epidemiological data emphasizing the relevance of housing conditions and exposure to hematophagous insects, such as bedbugs and kissing bugs of patients with the disease.…”

Section: Discussionmentioning

confidence: 50%

“…New foci in the Midwestern Brazilian States (Goiás, Mato Grosso, Mato Grosso do Sul) reported yearly incidences varying from 0.09 cases/10,000 inhabitants to 0.83 cases/10,000 inhabitants. 8 Frequency of 30.7 FS cases/ year through 1990 to 1999 in the State of Mato Grosso do Sul has been detected. 9 Endemic sites of PF were also found in other countries such as Colombia, Venezuela, Paraguay, and Peru.…”

Section: Introductionmentioning

confidence: 94%

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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“…O número de pacientes identificados no presente estudo em Antônio Pereira, também em busca ativa, revelou 4,57 casos para cada mil habitantes em acompanhamento na unidade básica de saúde local, valor que supera sobremaneira a prevalência estimada para o estado por Pimentel 8 . A não observação de disparidades em relação ao sexo está em conformidade com achados da literatura [9][10][11] , embora alguns autores admitam que há ligeira predominância do sexo feminino entre os portadores dessa forma de pênfigo 12 .…”

Section: ▄ Resultadosunclassified

“…Tradicionalmente, são descritas a exposição à picada de mosquito do gênero Simulium nigrimanum 13 (conhecido popularmente como borrachudo) e o pertencimento a região rural como fatores de risco associados ao PFE 12,14 . Entretanto, a relação entre vetores e possíveis fatores etiológicos ainda não é clara e pode incluir roedores e outras espécies de insetos hematófagos, bem como outras espécies do gênero Simulium, como S. pertinax 13 e S. pruinosum 9 .…”

Section: ▄ Discussãounclassified

Pênfigo Foliáceo Endêmico (Fogo Selvagem) e sua associação com fatores ambientais e ocupacionais em Ouro Preto, Minas Gerais, Brasil

et al. 2017

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Cad. Saúde Colet., 2017, Rio de Janeiro, 25 (2) ResumoPênfigo compreende grupo de doenças bolhosas autoimunes que possuem tendência à progressão, com evolução ilimitada e crônica e com prognóstico potencialmente fatal. O tipo mais comum é o Pênfigo Foliáceo Endêmico (PFE), caracterizado pela presença de lesões cutâneas com formação de bolhas na face, no couro cabeludo e na região interescapular. Fatores de ordem ambiental, genéticos e imunológicos podem desencadear a enfermidade. Entre os fatores ambientais, exposição a mercúrio, poeiras minerais e a picada do mosquito Simulium nigrimanum devem ser considerados. Buscou-se, neste artigo, relatar a ocorrência do PFE entre a população de Antônio Pereira, distrito rural de Ouro Preto, Minas Gerais, bem como identificar possíveis associações da doença com fatores ocupacionais e ambientais locais. Foi constatada uma prevalência de 4,57 casos de PFE/1.000 habitantes, considerada bastante elevada. Observou-se associação estatística entre a doença e contato com barragem de rejeitos de mineração (p = 0,048) e exposição ao mercúrio (p = 0,008). Os resultados indicam a necessidade de vigilância epidemiológica eficaz das comunidades afetadas, assim como adequada assistência à saúde dos pacientes acometidos pela doença. Palavras-chave: pênfigo foliáceo endêmico; fatores ambientais e ocupacionais; mineração; mercúrio; barragem de rejeito. AbstractPemphigus comprises a group of autoimmune bullous diseases, which have a tendency of progression, with unlimited and chronic development and with a potentially to fatal disease prognosis. The most common type is the Endemic Pemphigus Foliaceus (EPF), characterized by the presence of skin lesions with blistering located on the face, scalp and interscapular region. Environmental, Cad. Saúde Colet., 2017, Rio de Janeiro, 25 (2): 225-232 226

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Endemic Pemphigus foliaceus ("Fogo selvagem"): a series from the Northeastern region of the State of São Paulo, Brazil, 1973-1998

Abstract: the present data characterize the northeastern region of the state of São Paulo as a remaining endemic focus of EPF.

Cited by 36 publications

References 3 publications

Mast cells, Mast/Stem Cell Growth Factor receptor (c-kit/cd117) and IgE may be integral to the pathogenesis of endemic pemphigus foliaceus

Mast cells, Mast/Stem Cell Growth Factor receptor (c-kit/cd117) and IgE may be integral to the pathogenesis of endemic pemphigus foliaceus

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

Pênfigo Foliáceo Endêmico (Fogo Selvagem) e sua associação com fatores ambientais e ocupacionais em Ouro Preto, Minas Gerais, Brasil

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