Současný pohled na podávání intravenózních imunoglobulinů u pacientů s toxickou epidermální nekrolýzou
| Title in English | The present perspective on the administration of intravenous immunoglobulins in patients with toxic epidermal necrolysis |
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| Authors | |
| Year of publication | 2020 |
| Type | Article in Periodical |
| Magazine / Source | Klinická farmakologie a farmacie |
| MU Faculty or unit | |
| Citation | |
| Web | https://www.klinickafarmakologie.cz/pdfs/far/2020/03/06.pdf |
| Doi | http://dx.doi.org/10.36290/far.2020.024 |
| Keywords | toxic epidermal necrolysis; therapy; intravenous immunoglobulins; apoptosis; lethality |
| Description | Toxic epidermal necrolysis is a rare life-threatening disease dominantly manifesting on the skin but frequently affecting mucosa as well. The clinical symptomatology results from a pathophysiological process causing massive apoptosis of keratinocytes, dominantly those in the dermal-epidermal junction. This, in turn, leads to a formation of a bulla and a separation of practically the entire epidermis, exposing the dermis. Although the complex pathophysiological background of this disorder has not been fully clarified yet, the interaction at the level of the Fas ligand and Fas receptor is considered the dominant pathway leading to this condition. Intravenous immunoglobulins are among the most potent inhibitors of that bond and, in effect, of the subsequent transmission of the signal inducing the keratinocyte apoptosis. The presented review aims to clarify the reasons why the intravenous immunoglobulin therapy may sometimes fail. |