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 i nvolve the skin. Morbidity is associated with the propensity for scar forma tion and may be especially severe when mucosal surfaces such as the conjunc tivae, larynx, esophagus, or urethra are involved. On direct immunofluoresc ence microscopy, CP is characterized by the linear deposition of immunoreac tants, principally IgG and C3, along epithelial basement membranes. Over th e last 10 years, studies in a number of laboratories have shown that circul ating autoantibodies in patients with CP may target one of several differen t autoantigens. One subset of patients with the CP-phenotype have IgG anti- basement membrane autoantibodies against laminin 5 (alpha3 beta3 gamma2) (i .e., patients with anti-epiligrin CP [AECP]). This review discusses recent advances in the understanding of CP and emphasizes salient features of AECP pathophysiology.