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The pemphigoids, autoimmune blistering diseases of the skin and mucosae: immunopathogenic mechanisms, prognostic and diagnostic markers (PEMPHIGOIDS & IMMUNITY)

Objective

The project is aimed at a comprehensive analysis of the pathophysiology of a group of rare, but severe and chronic autoimmune bullous skin disorders, the pemphigoids (PEs), characterized by defective epithelial adhesion and autoantibodies against components of the skin basement membrane zone. These diseases represent a prototype of organ-specific autoimmune disorders which share common pathogenetic mechanisms. The objectives are: the analysis of molecular interactions and processing of epithelial adhesion proteins targets in the PEs; the characterization of the humoral immune responses and T cell immune responses in the PEs; the study of disease development risk factors. Expected results are:
(i) a better understanding of the immunopathogenesis of PEs disorders;
(ii) the definition of markers for disease severity, progression and outcome,
(iii) the identification of subjects at risk for disease,
(iv) the development of new diagnostic tools and of
(v) novel immunotherapy for these diseases. Altogether these results will also enhance understanding and allow development of new diagnostic and therapeutic approaches in other autoimmune disorders.

Funding Scheme

CSC - Cost-sharing contracts

Coordinator

PROVINCIA ITALIANA DELLA CONGREGAZIONE DEI FIGLI DELL'IMMACOLATA CONCEZIONE - ISTITUTO DERMOPATICO DELL'IMMACOLATA
Address
Via Dei Monti Di Creta 104
00167 Roma
Italy

Participants (4)

INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE
France
Address
Avenue De Valombrose 28
06107 Nice
UNIVERSITAETSKLINIKUM FREIBURG
Germany
Address
Hauptstrasse 7
79104 Freiburg (In Breisgau)
UNIVERSITY OF ERLANGEN-NUREMBERG
Germany
Address
14,Hartmannstrasse 14
91052 Erlangen
WIESLAB AB
Sweden
Address
Ideon Research Park
223 70 Lund