Chiapa M, Becker I

Language: Spanish
References: 0
Page: 100-108
PDF size: 216.69 Kb.

ABSTRACT

Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membranes. It is mediated by autoantibodies against desmoglein 3, an intercellular adhesion glycoprotein that forms part of the desmosomes. Loss of tolerance to this autoantigen happens for a still unknown reason; however, this disease has a very strong association with some human leukocyte antigen (HLA) molecules whose structure favours the binding of certain peptides of desmoglein 3. The presence of these HLA molecules would not be important if there were no desmoglein 3 reactive T lymphocytes, however, it has been reported that some people with these HLA molecules do have T and B cell clones reactive against desmoglein 3 circulating in their blood. Evidence indicates that, as opposed to healthy people, patients with pemphigus vulgaris have an altered regulation of their autoreactive lymphocytes, favouring autoantibody production. The mechanism of action of the autoantibodies has turned out to be more complicated than it was originally thought and is not well established yet. Nevertheless, in the last few years important discoveries have been made that point to a role of autoantibodies in starting different signalling cascades that lead to loss of adhesion between keratinocytes and death of these cells, resulting in acantholysis as histological manifestation and blisters as clinical manifestation. The present review addresses recent data made in the field.