The genetic basis of pemphigus vulgaris
Abstract The severe autoimmune blistering disease pemphigus vulgaris (PV) is most probably mainly due to autoantibodies directed against desmosomal components of the epithelium, disrupting cell‐cell adhesion. The precise mechanisms underlying the development of the disease are still unknown, and imm...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Wiley
2023-06-01
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| Series: | JEADV Clinical Practice |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/jvc2.129 |
| _version_ | 1797813991882358784 |
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| author | Dan Vodo Eli Sprecher |
| author_facet | Dan Vodo Eli Sprecher |
| author_sort | Dan Vodo |
| collection | DOAJ |
| description | Abstract The severe autoimmune blistering disease pemphigus vulgaris (PV) is most probably mainly due to autoantibodies directed against desmosomal components of the epithelium, disrupting cell‐cell adhesion. The precise mechanisms underlying the development of the disease are still unknown, and immunosuppressive medications such as corticosteroids, which are linked to potentially serious adverse effects, are still the mainstay of treatment. Ethnic susceptibility and familial incidence hint to a genetic component to the pathophysiology of PV, which, if identified, could further improve our understanding of PV pathogenesis and lead to the discovery of new therapeutic targets for this potentially fatal condition. In this article, we review the evidence supporting a genetic component in PV and discuss the clinical and therapeutic implications of these discoveries. |
| first_indexed | 2024-03-13T08:00:56Z |
| format | Article |
| id | doaj.art-3428e0ed239c4db3b90980f56865674d |
| institution | Directory Open Access Journal |
| issn | 2768-6566 |
| language | English |
| last_indexed | 2024-03-13T08:00:56Z |
| publishDate | 2023-06-01 |
| publisher | Wiley |
| record_format | Article |
| series | JEADV Clinical Practice |
| spelling | doaj.art-3428e0ed239c4db3b90980f56865674d2023-06-01T15:13:20ZengWileyJEADV Clinical Practice2768-65662023-06-012220321210.1002/jvc2.129The genetic basis of pemphigus vulgarisDan Vodo0Eli Sprecher1Division of Dermatology Tel Aviv Sourasky Medical Center Tel Aviv IsraelDivision of Dermatology Tel Aviv Sourasky Medical Center Tel Aviv IsraelAbstract The severe autoimmune blistering disease pemphigus vulgaris (PV) is most probably mainly due to autoantibodies directed against desmosomal components of the epithelium, disrupting cell‐cell adhesion. The precise mechanisms underlying the development of the disease are still unknown, and immunosuppressive medications such as corticosteroids, which are linked to potentially serious adverse effects, are still the mainstay of treatment. Ethnic susceptibility and familial incidence hint to a genetic component to the pathophysiology of PV, which, if identified, could further improve our understanding of PV pathogenesis and lead to the discovery of new therapeutic targets for this potentially fatal condition. In this article, we review the evidence supporting a genetic component in PV and discuss the clinical and therapeutic implications of these discoveries.https://doi.org/10.1002/jvc2.129geneticsHLApemphigusST18 |
| spellingShingle | Dan Vodo Eli Sprecher The genetic basis of pemphigus vulgaris JEADV Clinical Practice genetics HLA pemphigus ST18 |
| title | The genetic basis of pemphigus vulgaris |
| title_full | The genetic basis of pemphigus vulgaris |
| title_fullStr | The genetic basis of pemphigus vulgaris |
| title_full_unstemmed | The genetic basis of pemphigus vulgaris |
| title_short | The genetic basis of pemphigus vulgaris |
| title_sort | genetic basis of pemphigus vulgaris |
| topic | genetics HLA pemphigus ST18 |
| url | https://doi.org/10.1002/jvc2.129 |
| work_keys_str_mv | AT danvodo thegeneticbasisofpemphigusvulgaris AT elisprecher thegeneticbasisofpemphigusvulgaris AT danvodo geneticbasisofpemphigusvulgaris AT elisprecher geneticbasisofpemphigusvulgaris |