Stromal regulation of inflammation in pemphigoid diseases

Pemphigoid diseases (PDs) such as Bullous Pemphigoid or Epidermolysis bullosa acquisita (EBA) are caused by autoantibodies against structural proteins of the dermal-epidermal junction. PDs are characterized by tense blisters and erosions on skin or mucous membranes, exhibit a relapsing-remitting disease pattern and frequently flare upon changes in therapy. Furthermore, skin lesions exhibit a scattered regional distribution, affecting certain skin areas more often while others are spared. The preferential affection of specific body parts although antibody deposition is more or less homogenously distributed over the skin on the whole body suggests an involvement of local cell populations mediating inflammatory tissue priming.  

In a project within the SFB1526, we aim to delineate the priming response of the skin triggered by autoantibodies during iterated EBA in mice and focus on promising pathways that emerged to be differentially regulated in primed dermal fibroblasts to “un-prime” these cells and enable long term healing of PDs.  

Related publications: Friscic et al. (PMID 33761330)
Main responsible: Alessia Sbaraglia