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Autoantibodies in Pemphigus Foliaceus
Book chapter

Autoantibodies in Pemphigus Foliaceus

Agustin España-Alonso, Janet A. Fairley, George J. Giudice and Luis A. Diaz
Autoantibodies, pp.754-758
Elsevier Science B.V
1996
DOI: 10.1016/B978-044482383-0/50115-1

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Abstract

This chapter discusses the pathogenetic role, methods of detection, and clinical use of pemphigus foliaceus (PF) autoantibodies. Autoantibodies associated with PF were first identified by direct and indirect immunofluorescence techniques that showed that circulating autoantibodies from PF patients bind to the intercellular regions of human and monkey esophageal squamous epithelia and produce a characteristic staining pattern that is a diagnostic finding for all of the clinical variants of pemphigus. The pathogenic role of PF antiepidermal autoantibodies was demonstrated in vitro when patients' immunoglobulin (Ig) G, introduced into the growth medium of epidermal cell cultures and skin organ cultures, caused acantholysis and increased activity of plasminogen activator. PF autoantibodies show an apparent clonal restriction as evidenced by isoelectric focusing analysis. The endemic Fogo Selvagem (FS) and nonendemic forms of PF are unique examples of human organ-specific humoral autoimmune diseases. Apparently, PF autoantibodies, binding to extracellular epitopes of desmosomal glycoprotein, impair its adhesive function and cause epidermal cell-cell detachment and intraepidermal blister formation. Binding of PF autoantibodies is postulated to activate plasminogen activator from epidermal cells, with resultant keratinocyte detachment and blistering.

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