Acantholysis in pemphigus

Gerda van der Wier

    Research output: ThesisThesis fully internal (DIV)

    1861 Downloads (Pure)

    Abstract

    Pemphigus is disease in which patients have blisters of the skin and the mucous membranes. There are different forms of pemphigus. Patients with pemphigus foliaceus (PF) have superficial blistering of the skin. Patients with pemphigus vulgaris (PV) have deeper blistering of the skin (mucosal-dominant PV) or they have blisters of both the skin and the mucous membranes (mucocutaneous PV).

    Patients with pemphigus produce antibodies directed against the structures (desmosomes) that attach the skin cells together en give the skin strength. Desmosomes are composed of several proteins, of which the most important are desmoglein 1 and desmoglein 3. In PF the antibodies are directed against desmoglein 1. Patients with mucosal-dominant PV produce antibodies directed against desmoglein 3. Mucocutaneous PV is characterized by antibodies directed against both desmoglein 1 and 3.

    In the past years different theories have been described that try to explain the mechanism of blistering in pemphigus. The goal of this thesis is to gain more insight into the mechanism of blistering in pemphigus.

    In skin of PF patients, clusters are present composed of desmoglein 1 and in skin of mucosal-dominant PV the clusters are composed of desmoglein 3. Clusters composed of both desmoglein 1 and 3 are present in skin of mucocutaneous PV patients. Electronmicroscopy shows that the desmosomes in patient skin become smaller. We think that the desmogleïnes are attacked by the antibodies, form clusters and are no longer able to be assembled into desmosomes. The desmosomes become smaller, which leads to fragile skin and eventually to blistering.
    Translated title of the contributionAcantholyse in pemphigus
    Original languageEnglish
    QualificationDoctor of Philosophy
    Awarding Institution
    • University of Groningen
    Supervisors/Advisors
    • Jonkman, Marcellinus, Supervisor
    • Pas, Hendri, Co-supervisor
    • Diercks, Gilles, Co-supervisor
    Award date5-Nov-2014
    Place of Publication[S.l.]
    Publisher
    Print ISBNs978-90-367-7296-9
    Electronic ISBNs978-90-367-7295-2
    Publication statusPublished - 2014

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