Pemphigus pathogenesis. Insights from patient skin studies

PhD ceremony: Ms. D.A.M. Oktarina, 11.00 uur, Academiegebouw, Broerstraat 5, Groningen

Thesis: Pemphigus pathogenesis. Insights from patient skin studies

Promotor(s): prof. M.F. Jonkman

Faculty: Medical Sciences

 

Pemphigus (blister disease) patient skins show that there is a shift in the distribution of desmoglein 1 and 3 (autoantigens of pemphigus), and to a lesser extent, plakoglobin (intracytoplasmic protein), separated from the other components in desmosome (structures which form a solid binding between epithelial skin cells). The binding of pathogenic IgG to desmogleins thus interferes with the dynamics of the desmosomal turnover because newly synthesized desmogleins to which IgG is bound can no longer be incorporated into desmosomes, and making them 'melt away'. From these results we propose a concept that the pathogenesis of acantholysis (cel-cel separation) in pemphigus is explained by the desmosomal desmoglein depletion (non-assembly desmosomal depletion hypothesis).