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A Journal on Dermatology and Sexually Transmitted Diseases

Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
Indexed/Abstracted in: EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 1,014

Frequency: Bi-Monthly

ISSN 0392-0488

Online ISSN 1827-1820


Giornale Italiano di Dermatologia e Venereologia 2007 August;142(4):353-62



T cell involvement in pemphigus

Gilbert D. 1, Mouquet H. 1, Joly P. 1,2

1 INSERM U519, Federate Institute for Multidisciplinary Research on Peptides, Faculty of Medicine and Pharmacology Rouen, France
2 Department of Dermatology Charles Nicolle University Hospital, Rouen, France

Pemphigus are rare organ-specific autoimmune blistering diseases, characterized by pathogenic autoantibodies directed against desmogleins, specific desmosomal transmembrane glycoproteins. Binding of autoantibodies to their targets induces a loss of adhesion between keratinocytes resulting in blister formation. There are many arguments to consider that both induction and regulation of autoantibody production need the activation of specific autoreactive T cells. At first, the major histocompatibility complex, in particular the class II locus was demonstrated to be associated with pemphigus. Subsequently, the activation of autoreactive T cells by the extracellular domain of Dsg1 and Dsg3 was demonstrated in pemphigus foliaceus (PF) and pemphigus vulgaris (PV) patients respectively. The T cell recognition of different Dsg3 peptides was restricted by HLA class II molecules and was also obtained in healthy individuals who carried PV-associated HLA class II alleles. Both Th1 and Th2 T cell clones were detected in PV patients, at different stages of the disease and the ratio Th1/Th2 was shown to be related to the anti-Dsg3 autoantibody titer. Interestingly, healthy subjects exclusively exhibited Th1 reactivity against Dsg3. Furthermore, the presence of Type 1 regulatory cells (Tr1) was mainly found in healthy subjects, suggesting that an imbalance between Th1, Th2 and Tr1 cells may be critical for the tolerance breakdown observed in pemphigus patients.

language: English


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