Oral
pemphigoid (OP) is a chronic
autoimmune disease, involving the oral cavity, characterized by a homogenous linear deposition of
immunoglobulins,
complement, or both along the basement membrane zone (BMZ) and a subepithelial
blister formation. The alpha6/beta4 heterodimer is an
integrin family of
adhesion receptors, which mediates basal cell to matrix interactions. Recent evidence suggests a pathophysiologic role for
antibodies against human
alpha6 integrin in
blister formation in OP, in organ culture studies. Fifty percent of OP patients have been reported to experience
disease progression to involve other mucosal tissues, including the eye and larynx. To prevent this extension of disease, systemic
therapy with systemic
corticosteroids,
dapsone, and
immunosuppressive agents has been recommended. The use of
intravenous immunoglobulin (
IVIg) in the treatment of
pemphigoid has been recently described. In this study, we present the use of
IVIg, in a group of seven patients, with severe OP, in whom systemic conventional treatment was contraindicated. To determine the influence of treatment on
antibodies to human
alpha6 integrin in OP, seven patients with OP treated with
IVIg therapy and a comparable control group of seven patients with OP, treated with conventional
therapy, were evaluated at monthly intervals, for a 12 consecutive month treatment period. An effective clinical response was observed in all seven patients treated with
IVIg therapy, after a mean treatment period of 4.5 months.
IVIg therapy induced a prolonged and sustained clinical remission in all seven patients after a mean treatment period of 26.9 months. A statistically significant difference was observed in the quality of life pre- and post-
IVIg therapy (P < 0.001). Both the study and the control groups had a very similar initial serological response to treatment. A statistically significant reduction in the antibody titres was observed after four months of treatment, in both groups (P = 0.015). Thereafter, patients treated with
IVIg therapy had a faster rate of decline in the antibody titres, and the difference in the rate of decline between the study and control groups became statistically significant after six months of treatment (P = 0.03). The use of
IVIg therapy resulted in reduction of antialpha6 antibody titres and in inducing and maintaining both a sustained, clinical and serological remission.