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• W2094204452 abstract "Pemphigus is a rare organ-specific autoimmune blistering disease involving skin and mucous membrane. Acantholysis (i.e., loss of adhesion between keratinocytes) is responsible for intraepidermal cleavage and is mediated by pathogenic autoantibodies directed against desmosomal proteins. Recently, highly significant progress has been made in the understanding of the pathogenesis of pemphigus. The present issue summarizes this progress and focuses on diagnosis and management of pemphigus patients. The different target auto-antigens are well characterized, and their identification allows immunological classification of the different types of pemphigus. Production of recombinant proteins led to the development of ELISA tests, which will soon become available for routine diagnosis. Furthermore, the use of these recombinant antigens may provide an interesting approach in the development of a specific immunotherapy of pemphigus patients. At the same time, new pemphigus variants have been described and better characterized, including paraneoplastic pemphigus, IgA pemphigus, and herpetiform pemphigus; however, the basis for the immunological tolerance breakdown against desmosomal proteins remains unknown. Genetic factors are suggested by the strong association of the disease with particular HLA class II molecules. Their ability to present desmoglein-derived peptides to specific T-cell clones has been confirmed and emphasizes the key role of CD4 T lymphocytes in the production of pathogenic antibodies. Genetic factors, however, are probably not exclusive, and pemphigus probably also involves environmental factors that remain to be clearly individualized. Therefore, although pemphigus is a rare disease, it constitutes one of the best model for studying organ-specific autoimmunity. The first part of this article will focus on pemphigus antibodies: their identification, the models used to ascertain their pathogenic properties, and the putative mechanisms of acantholysis. In the second part, the different target antigens involved in the various types of pemphigus will be described. Finally, the mechanisms possibly involved in the tolerance breakdown and antibody production will be discussed." @default.
• W2094204452 created "2016-06-24" @default.
• W2094204452 creator A5017289601 @default.
• W2094204452 creator A5050985511 @default.
• W2094204452 date "2001-11-01" @default.
• W2094204452 modified "2023-10-03" @default.
• W2094204452 title "Pemphigus: autoimmune diseases of keratinocyte’s adhesion molecules" @default.
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• W2094204452 doi "https://doi.org/10.1016/s0738-081x(00)00191-7" @default.
• W2094204452 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/11705674" @default.
• W2094204452 hasPublicationYear "2001" @default.

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