Pemphigus

M Kasperkiewicz, CT Ellebrecht, H Takahashi… - Nature reviews Disease …, 2017 - nature.com
Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous
epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of cell adhesion)
causes blisters and erosions. Pemphigus has three major subtypes: pemphigus vulgaris,
pemphigus foliaceus and paraneoplastic pemphigus. IgG autoantibodies are
characteristically raised against desmoglein 1 and desmoglein 3, which are cell–cell
adhesion molecules found in desmosomes. The sites of blister formation can be …

Pemphigus

E Schmidt, M Kasperkiewicz, P Joly - The Lancet, 2019 - thelancet.com
Pemphigus consists of a group of rare and severe autoimmune blistering diseases mediated
by pathogenic autoantibodies mainly directed against two desmosomal adhesion proteins,
desmoglein (Dsg) 1 and Dsg3 (also known as DG1 and DG3), which are present in the skin
and surface-close mucosae. The binding of autoantibodies to Dsg proteins induces a
separation of neighbouring keratinocytes, in a process known as acantholysis. The two main
pemphigus variants are pemphigus vulgaris, which often originates with painful oral …