Antiepiligrin cicatricial pemphigoid - An underdiagnosed entity within thespectrum of scarring autoimmune subepidermal bullous diseases?

Background: Antiepiligrin cicatricial pemphigoid (AECP) is a chronic autoim mune subepidermal blistering disease characterized by autoantibodies to lam inin 5 and clinical features of cicatricial pemphigoid. Only a few patients with AECP have been described to date. The aim of the present study was to analyze the relative frequency of AECP among patients with the clinical ph enotype of cicatricial pemphigoid. Observations: Serum from 16 consecutive patients with the clinical phenotyp e of cicatricial pemphigoid were included in this study. Nine patients had circulating IgG autoantibodies by indirect immunofluorescence on sodium chl oride-split skin; patients' IgG bound to the epidermal side (n = 2), dermal side (n = 5), or both sides (n = 2) of this test substrate. Interestingly, all 5 cases with dermal binding immunoprecipitated laminin 5 from extracts and media of cultured keratinocytes, and 4 of these serum samples reacted with the alpha 3 subunit of laminin 5 by immunoblotting. None of the patien ts with dermal binding of IgG demonstrated autoantibodies to type VII colla gen. Conclusion: Our data suggest that, among patients with the clinical phenoty pe of cicatricial pemphigoid, AECP may be more frequent than previously ass umed.