Cells and Immunoglobulins

Probably the best example for an immunoglobulin-mediated disease is pemphigus vugaris. In patients, this disease is associated with little inflammation and seems to be directly mediated by the binding of autoreactive immunoglobulins to the desmogleins that guarantee the adherence between kerati-nocytes (Amagai et al. 1991). Indeed, transfer of patient sera and monoclonal antibodies directed against desmoglein 3 into the skin of new-born mice can directly induce acanthosis (Rock et al. 1989). This critical role for a direct binding of immunoglobulins to desmoglein structures is further supported by the observation that in patients with pemphigus vugaris the disease activity correlates closely with the serum levels of the autoantibodies (Hertl 2000). Such a close association is unusual for other autoimmune diseases, including lupus erythematosus or bullous pemphigoid. For comparisons, bullous pemphigoid is of special interest. It is also an immunoglobulin mediated bullous skin disease. In sharp contrast to pemphigus vulgaris, the clinical manifestation of bullous pemphigoid does not only require deposition of autoantigens but also an inflammatory milieu that causes detachment of the basement membrane (Liu et al. 1998; Liu et al. 2000). Consequently, transfer of specific sera or immunoglobulins under the skin of new born nude mice alone is not sufficient for the induction of blisters. It requires, in addition, activation of the complement cascade and inflammation, involving the recruitment of granulocytes (Liu et al. 1995).

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