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An Underdiagnosed Entity Within the Spectrum of Scarring Autoimmune Subepidermal Bullous Diseases?

Martin Leverkus, MD; Enno Schmidt, MD; Zelmira Lazarova, MD; Eva-Bettina Bröcker, MD; Kim B. Yancey, MD; Detlef Zillikens, MD

Arch Dermatol. 1999;135:1091-1098.

Background  Antiepiligrin cicatricial pemphigoid (AECP) is a chronic autoimmune subepidermal blistering disease characterized by autoantibodies to laminin 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 phenotype of cicatricial pemphigoid.

Observations  Serum from 16 consecutive patients with the clinical phenotype of cicatricial pemphigoid were included in this study. Nine patients had circulating IgG autoantibodies by indirect immunofluorescence on sodium chloride–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 3 subunit of laminin 5 by immunoblotting. None of the patients with dermal binding of IgG demonstrated autoantibodies to type VII collagen.

Conclusion  Our data suggest that, among patients with the clinical phenotype of cicatricial pemphigoid, AECP may be more frequent than previously assumed.

From the Department of Dermatology, University of Würzburg Medical School, Würzburg, Germany (Drs Leverkus, Schmidt, Bröcker, and Zillikens); and Dermatology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Md (Drs Lazarova and Yancey).

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