Solimar Ribeiro Carlete Filho, Luana Amorim Morais da Silva, Caio Rodrigues Maia, Paulo Roberto de Andrade Santos, Pollianna Muniz Alves, Pedro Paulo de Andrade Santos
{"title":"Oral pemphigus vulgaris diagnostic characteristics and treatment: a systematic review.","authors":"Solimar Ribeiro Carlete Filho, Luana Amorim Morais da Silva, Caio Rodrigues Maia, Paulo Roberto de Andrade Santos, Pollianna Muniz Alves, Pedro Paulo de Andrade Santos","doi":"10.1007/s00795-024-00414-y","DOIUrl":null,"url":null,"abstract":"<p><p>Pemphigus vulgaris (PV) is a rare, potentially fatal, immune-mediated chronic disease characterized by the presence of bullous intraepithelial lesions on mucous membranes and skin. This study aimed to perform a systematic literature review covering PV clinical and histopathological aspects and treatment. The literature searches were carried out in the Pubmed, Periódicos Capes, Scopus, Science Direct, Web of Science and Scielo databases. Articles in English or Spanish published from 2000 to 2022 comprising case reports, case series and literature reviews with case report were included. After the analyses, 21 articles were selected. PV generally presents in the third to sixth decades of life and exhibits no gender predilection. The disease manifests itself clinically through irregular and painful blisters that rupture, resulting in erosion and ulceration areas. Histopathologically, the presence of an intraepithelial cleft located above the basal layer and acantholysis are observed. Standard treatment encompasses systemic and topical corticosteroids, with prednisolone being widely employed. Management consists of a remission induction phase and a maintenance phase. An early and accurate diagnosis is paramount to quickly initiate treatment, resulting in more favorable prognoses, as the choice of treatment and responses depend on the severity of the disease. Registered at the International Prospective Register of Systematic Reviews (PROSPERO): Number CRD42024497313.</p>","PeriodicalId":18338,"journal":{"name":"Medical Molecular Morphology","volume":" ","pages":""},"PeriodicalIF":1.2000,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Medical Molecular Morphology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00795-024-00414-y","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"PATHOLOGY","Score":null,"Total":0}
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Abstract
Pemphigus vulgaris (PV) is a rare, potentially fatal, immune-mediated chronic disease characterized by the presence of bullous intraepithelial lesions on mucous membranes and skin. This study aimed to perform a systematic literature review covering PV clinical and histopathological aspects and treatment. The literature searches were carried out in the Pubmed, Periódicos Capes, Scopus, Science Direct, Web of Science and Scielo databases. Articles in English or Spanish published from 2000 to 2022 comprising case reports, case series and literature reviews with case report were included. After the analyses, 21 articles were selected. PV generally presents in the third to sixth decades of life and exhibits no gender predilection. The disease manifests itself clinically through irregular and painful blisters that rupture, resulting in erosion and ulceration areas. Histopathologically, the presence of an intraepithelial cleft located above the basal layer and acantholysis are observed. Standard treatment encompasses systemic and topical corticosteroids, with prednisolone being widely employed. Management consists of a remission induction phase and a maintenance phase. An early and accurate diagnosis is paramount to quickly initiate treatment, resulting in more favorable prognoses, as the choice of treatment and responses depend on the severity of the disease. Registered at the International Prospective Register of Systematic Reviews (PROSPERO): Number CRD42024497313.
期刊介绍：
Medical Molecular Morphology is an international forum for researchers in both basic and clinical medicine to present and discuss new research on the structural mechanisms and the processes of health and disease at the molecular level. The structures of molecules, organelles, cells, tissues, and organs determine their normal function. Disease is thus best understood in terms of structural changes in these different levels of biological organization, especially in molecules and molecular interactions as well as the cellular localization of chemical components. Medical Molecular Morphology welcomes articles on basic or clinical research in the fields of cell biology, molecular biology, and medical, veterinary, and dental sciences using techniques for structural research such as electron microscopy, confocal laser scanning microscopy, enzyme histochemistry, immunohistochemistry, radioautography, X-ray microanalysis, and in situ hybridization.
Manuscripts submitted for publication must contain a statement to the effect that all human studies have been reviewed by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in an appropriate version of the 1964 Declaration of Helsinki. It should also be stated clearly in the text that all persons gave their informed consent prior to their inclusion in the study. Details that might disclose the identity of the subjects under study should be omitted.
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