Fogo selvagem: endemic pemphigus foliaceus

Hans-Filho, Günter; Aoki, Valéria; Bittner, Nelise Ritter Hans; Bittner, Guilherme Canho

doi:10.1590/abd1806-4841.20188235

Cited by 28 publications

(23 citation statements)

References 57 publications

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“…Despite of the limited patients’ number in our study, both groups were considered homogeneous relative to age, gender, skin color, and residence type. This homogeneity between patient groups was probably of the Brazilian epidemiological and environmental factors cited in previous study of our group and others studies (Hans-Filho et al, 2018; Timóteo et al, 2010). Clinicals characteristics as medical treatment time, concomitant medication dosage and cutaneous involvement did not present correlations between the patients groups studied.…”

Section: Discussionsupporting

confidence: 76%

Pemphigus foliaceus patients (Fogo Selvagem) treated with kinesiotherapy presented lower levels of proinflammatory cytokines

et al. 2019

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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. Understanding the impact of such methodology in immunology of the FS, may constitute an alternative and effective approach. We compare the levels of serum cytokines and chemokines between nine patients with FS submitted to kinesiotherapy for 12 weeks and ten patients not submitted to kinesiotherapy. The kinesiotherapy was composed by self-stretching followed by a resistance training for upper and lower limbs. The protocol was carried out in three sections of eight to ten repetitions with 70% of the maximum load measured by test maximum of ten repetitions. After strengthening period patients performed a passive stretching. The training sessions lasted 50 min and were performed 3 times a week at least 12 weeks. Cytokines and chemokines were assessed in plasma using enzyme-linked immunosorbent assay and/or cytometric bead array. Patients with FS were being kinesiotherapy presented minors levels of interferon-γ, interleukin (IL)-17, IL-22, and IL-15 when compared to those not submitted to kinesiotherapy. No differences were observed for the detection of the chemokines chemokine ligand (CCL)-2, CCL-3, CCL-5, CCL-11, C-X-C motif chemokine 8 (CXCL-8), and CXCL-10. These results suggest that kinesiotherapy had a positive impact on inflammatory markers that are associated with disease worsening in FS.

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Section: Discussionsupporting

confidence: 76%

Pemphigus foliaceus patients (Fogo Selvagem) treated with kinesiotherapy presented lower levels of proinflammatory cytokines

et al. 2019

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“…Based on the desmoglein compensation theory, Dsg-1 expression is more intense in the upper portions of the epidermis, whereas in PV, Dsg-3 predominates in the lower layers of the epidermis and in mucous membranes. 9,95-102…”

Section: Pemphigus Foliaceusmentioning

confidence: 99%

Consensus on the treatment of autoimmune bullous dermatoses: pemphigus vulgaris and pemphigus foliaceus - Brazilian Society of Dermatology

Porro

Filho

Santi

2019

An. Bras. Dermatol.

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Pemphigus are intraepidermal autoimmune bullous dermatoses that occur with lesions on the skin and / or mucous membranes. The most frequent types are pemphigus vulgaris and pemphigus foliaceus (classic and endemic). This consensus aims to present a complete and updated review of the treatment of these two more frequent forms of pemphigus, based on the literature and the personal experience of the authors. In moderate and severe cases of pemphigus vulgaris and foliaceus, systemic corticosteroid therapy (prednisone or prednisolone) is the treatment of choice. Adjuvant drugs, usually immunosuppressive drugs (azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide) may be prescribed as corticosteroid sparers in refractory cases or with contraindications to corticosteroids to minimize side effects. In severe and nonresponsive cases, corticosteroids in the form of intravenous pulse therapy, immunoglobulin and plasmapheresis / immunoadsorption can be administered. Immunobiological drugs, particularly rituximab, appear as a promising alternative. For milder cases, smaller doses of oral corticosteroid, dapsone and topical corticosteroids are options. At the end flowcharts are presented as suggestions for a therapeutic approach for patients with pemphigus vulgaris and pemphigus foliaceus.

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“…Some emerging therapies that have shown positive outcomes in other autoimmune diseases are being investigated ( Ruocco et al. , 2013 ; Kasperkiewicz et al , 2017 ; Hans-Filho et al , 2018 ; Yanovsky et al , 2019 ).…”

Section: Introductionmentioning

confidence: 99%

Beyond the HLA polymorphism: A complex pattern of genetic susceptibility to pemphigus

Petzl-Erler

2020

Genet. Mol. Biol.

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Pemphigus is a group of autoimmune bullous skin diseases that result in significant morbidity. As for other multifactorial autoimmune disorders, environmental factors may trigger the disease in genetically susceptible individuals. The goals of this review are to summarize the state of knowledge about the genetic variation that may affect the susceptibility and pathogenesis of pemphigus vulgaris and pemphigus foliaceus-both the endemic and the sporadic forms-, to compare and discuss the possible meaning of the associations reported, and to propose recommendations for new research initiatives. Understanding how genetic variants translate into pathogenic mechanisms and phenotypes remains a mystery for most of the polymorphisms that contribute to disease susceptibility. However, genetic studies provide a strong foundation for further developments in this field by generating testable hypotheses. Currently, results still have limited influence on disease prevention and prognosis, drug development, and clinical practice, although the perspectives for future applications for the benefit of patients are encouraging. Recommendations for the continued advancement of our understanding as to the impact of genetic variation on pemphigus include these partially overlapping goals: (1) Querying the functional effect of genetic variants on the regulation of gene expression through their impact on the nucleotide sequence of cis regulatory DNA elements such as promoters and enhancers, the splicing of RNA, the structure of regulatory RNAs and proteins, binding of these regulatory molecules to regulatory DNA elements, and alteration of epigenetic marks; (2) identifying key cell types and cell states that are implicated in pemphigus pathogenesis and explore their functional genomes; (3) integrating structural and functional genomics data; (4) performing disease-progression longitudinal studies to disclose the causal relationships between genetic and epigenetic variation and intermediate disease phenotypes; (5) understanding the influence of genetic and epigenetic variation in the response to treatment and the severity of the disease; (6) exploring gene-gene and genotype-environment interactions; (7) developing improved pemphigus-prone and non-prone animal models that are appropriate for research about the mechanisms that link genotypes to pemphigus. Achieving these goals will demand larger samples of patients and controls and multisite collaborations.

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Fogo selvagem: endemic pemphigus foliaceus

Cited by 28 publications

References 57 publications

Pemphigus foliaceus patients (Fogo Selvagem) treated with kinesiotherapy presented lower levels of proinflammatory cytokines

Pemphigus foliaceus patients (Fogo Selvagem) treated with kinesiotherapy presented lower levels of proinflammatory cytokines

Consensus on the treatment of autoimmune bullous dermatoses: pemphigus vulgaris and pemphigus foliaceus - Brazilian Society of Dermatology

Beyond the HLA polymorphism: A complex pattern of genetic susceptibility to pemphigus

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