Pathogenesis

 

The current understanding is that the HLA-mediated recognition of a certain drug or its metabolites by T cells induces an adaptive immune reaction directed against this agent.

 

Through – only partly understood mechanisms – involving an interplay between antigen presenting- and immune effector-cells, a strong production of cytotoxic molecules (FasL in keratinocytes, granulysin and annexin A1 in cytotoxic T cells, NK cells, NKT cells and monocytes) occurs within cells localized in the target organs (skin, mucous membranes), leading to the onset of massive apoptosis and necroptosis of epithelial cells, with subsequent epidermal/mucosal necrosis (necrolysis) and detachment, the cardinal clinical features of SJS/TEN. 

The IRTEN prospective registry with collection (optional) biological sample material (blood, small skin biopsy, DNA) for research purposes should provide interested researchers worldwide with high-quality patient-derived material to work towards improving our understanding of the pathogenesis of SJS-TEN.