The clinical and immunopathological manifestations of anti-epiligrin cicatricial pemphigoid, a recently defined subepithelial autoimmune blistering disease

Cicatricial pemphigoid (CP) is a rare, acquired, autoimmune, subepithelial blistering disease. It primarily affects mucous membranes but it also may involve the skin. Morbidity is associated with the propensity for scar formation and may be especially severe when mucosal surfaces such as the conjunctivae, larynx, esophagus, or urethra are involved. On direct immuno-fluorescence microscopy, CP is characterized by the linear deposition of immunoreactants, principally IgG and C3, along epithelial basement memnbranes. Over the last 10 years, studies in a number of laboratories have shown that circulating autoantibodies in patients with CP may target one of several different autoantigens. One subset of patients with the CP-phenotype have IgG anti-basement membrane autoantibodies against laminin 5 (α3β3γ2) (i.e., patients with anti-epiligrin CP [AECP]). This review disncusses recent advances in the understanding of CP and emphasizes salient features of AECP pathophysiology.