argenx SE Investor Day Presentation Deck
Bullous Pemphigoid Autoantibodies are Origin of Blister
Formation
EPIDERMIS
DERMIS
IL-6, IL-8
Efgartigimod
argenx
MECHANICAL
DISRUPTION
Autoantibodies
B Cells
сссс
COMPLEMENT
ACTIVATION
Chemokines &
proteases
Mast cells
3
Macrophages
4
Chemokines
INFLAMMATORY
REACTION
Neutrophils
T cell
Proteases
ROS
5
Eosinophils
Drawn after Schmidt E, Zillikens D. Lancet. 2013; Fang H, Li Q, Wang G. Autoimmun Rev. 2020
6
BLISTER
FORMATION
• BP180 and BP230: key pathogenic
hemidesmosomal antigens driving
blistering between epidermis and
dermis
• Autoantibody actions:
• Mechanical disruption of
keratinocyte adhesion
IgG autoantibody deposition
triggers complement activation
• Recruitment and activation of
immune cells (inflammatory cell
recruitment, release of
proteolytic enzymes)
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