The Pathophysiological Significance of Nondesmoglein Targets of Pemphigus Autoimmunity

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Abstract
SKIN BLISTERS occur in a variety of dermatologic conditions. Blisters in pemphigus are associated with the binding of IgG autoantibodies to keratinocyte cell-surface molecules.1 These "intercellular" or "pemphigus" antibodies bind to keratinocyte desmosomes and to desmosome-free areas of the keratinocyte cell membrane.2,3 Intercellular spaces enlarge, desmosomes decrease in number and eventually disappear, and the cells round up and detach from one another without cell death.4-7 This phenomenon is called "acantholysis." Use of high doses of glucocorticosteroids (prednisone, sometimes up to 1000 mg/d8) is required to suppress acantholysis. Corticosteroids are the mainstream of pemphigus management, but use of these drugs can cause severe adverse effects and complications, including death.9,10 The development of nonhormonal treatment of patients with pemphigus is pending elucidation of the pathophysiological pathways mediating acantholysis.