The Enigmatic Autoimmune Response in Endemic Pemphigus Foliaceus

Flores, Gustavo Kourí; Qian, Yuping; Díaz, Luis A.

doi:10.1016/s0001-7310(09)73377-0

Cited by 13 publications

(9 citation statements)

References 80 publications

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“…The presence of subcorneal acantholysis and intercellular staining demonstrated by direct immunofluorescence confirmed the diagnosis of PF: the histopathological features of bullous impetigo may be similar to those of PF, but immunopathology would be negative in bullous impetigo. 2 In PF, ELISA testing of antidesmoglein-1 antibodies is usually positive, with sensitivities as high as 97.9%, 13 and this test has been found to have greater sensitivity and specificity than conventional indirect immunofluorescence (IIF). 14 A positive ELISA was reported in two of the published cases of localized PF, and IIF results in these cases were variable (see Table 1).…”

Section: Clinical Casementioning

confidence: 99%

“…1,2 Disruption of cell---cell adhesion causes acantholysis resulting in superficial, fragile blisters that are prone to rupture. Consequently, patients typically present with erosions rather than blisters.…”

Section: Introductionmentioning

confidence: 99%

“…3 The pathogenesis of PF is not completely understood, but environmental exposure appears to be involved. 2,4,5 We present an interesting case of a young woman with a difficult-to-treat, localized form of PF that was unrelated to any known triggers. …”

Section: Introductionmentioning

confidence: 99%

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Localized Pemphigus Foliaceus with Unilateral Facial Involvement

Miner

Nousari

Alonso‐Llamazares

2014

Actas Dermo-Sifiliográficas

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Pemphigus foliaceus is a superficial vesiculobullous disease that typically presents with widespread lesions. Localized presentations are less frequent, and they typically occur in middle-aged patients, following exposure to topical medications, and later on, become more disseminated. We present a case of a 19-year-old female with a localized presentation of pemphigus foliaceus unrelated to previous topical medications, that was a diagnostic and therapeutically challenging case. We also discuss the literature on localized cases, differences in presentations and responses to various treatment modalities. Pénfigo foliáceo; Localizado; Enfermedades ampollosasPénfigo foliáceo localizado con afectación facial unilateral Resumen El pénfigo foliáceo es una enfermedad vesículo-ampollosa superficial caracterizada por la aparición de lesiones generalizadas. Las presentaciones localizadas son menos frecuentes y suelen observarse en pacientes de mediana edad tras la exposición a medicamentos tópicos que posteriormente evolucionan a formas más diseminadas. Presentamos el caso de una mujer de 19 años de edad con pénfigo foliáceo localizado no asociado a medicamentos tópicos previos cuyo diagnóstico y tratamiento han supuesto un reto. También analizamos la literatura existente sobre los casos de pénfigo foliáceo localizado, las diferencias en las presentaciones clínicas y las respuestas a distintos tipos de tratamientos.

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Section: Clinical Casementioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Localized Pemphigus Foliaceus with Unilateral Facial Involvement

Miner

Nousari

Alonso‐Llamazares

2014

Actas Dermo-Sifiliográficas

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“…The disease exhibits a strong association with the HLA-DRB1*0102, 0404 and 1402 alleles (Moraes et al , 1997). It is thought that an environmental antigen(s) may trigger FS (Flores et al , 2009). …”

Section: Introductionmentioning

confidence: 99%

IgG Autoantibody Response against Keratinocyte Cadherins in Endemic Pemphigus Foliaceus (Fogo Selvagem)

Flores

Culton

Prisayanh

et al. 2012

Journal of Investigative Dermatology

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It is well established that autoantibodies against desmoglein 3 and desmoglein 1 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 if FS patients and normal individuals living in endemic areas possess autoantibodies against other desmosomal cadherins and E-cadherin. Testing a large number of FS and endemic control sera by ELISA we find a consistent and specific autoantibody response against desmoglein 1 and other keratinocyte cadherins in these individuals, which is quite different from US controls. Overall, the highest correlations among the autoantibody responses tested are in the endemic controls, followed by FS patients, and lowest in the US controls. These findings suggest that multiple, perhaps cross reactive, keratinocyte cadherins are recognized by FS patients and endemic controls.

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“…As previously described in Peru, we observed a significant association between living in rural conditions with farming as main occupation and the presence of Abs against Dsg1 and/or the development of endemic PF, suggesting that this activity may expose individuals living in the endemic areas to particular environmental factors present in these south regions. In this regard, if the climate conditions in south Tunisia are quite different from those observed in South American endemic regions, the living conditions of the target populations are very similar because they are in close contact with animals and have farming activities …”

Section: Environmental Factorsmentioning

confidence: 99%

Update on immunogenetics of Tunisian endemic pemphigus foliaceus

Masmoudi

Abida

Masmoudi

et al. 2018

Journal of Leukocyte Biology

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Pemphigus foliaceus (PF) is an autoimmune blistering skin disease characterized by the presence of bullous skin lesions, the absence of mucous tissue involvement, and the production of autoantibodies directed against a keratinocyte transmembrane protein localized in the desmosome and member of the cadherines, desmoglein 1. These pathogenic auto-antibodies are responsible for the intra-epidermal formation of blisters through the loss of keratinocyte adhesion, the socalled acantholysis process. The endemic form of PF observed in the south of Tunisia is characterized by a significantly higher incidence rate compared to the sporadic form in northern countries, occurrence mainly in young women and the absence of cases during childhood. Tunisian endemic PF is an ideal research model for the decryption of the puzzle of genetic and environmental factors and their interactions in the development of autoimmune diseases. In this review, we will summarize recent findings regarding the epidemiologic and immunologic features of Tunisian PF and its genetic and environmental factors. K E Y W O R D Sautoimmune blistering disease, desmoglein, genetic polymorphism, HLA, Prolactine, TLR

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The Enigmatic Autoimmune Response in Endemic Pemphigus Foliaceus

Cited by 13 publications

References 80 publications

Localized Pemphigus Foliaceus with Unilateral Facial Involvement

Localized Pemphigus Foliaceus with Unilateral Facial Involvement

IgG Autoantibody Response against Keratinocyte Cadherins in Endemic Pemphigus Foliaceus (Fogo Selvagem)

Update on immunogenetics of Tunisian endemic pemphigus foliaceus

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