Anti-epiligrin cicatricial pemphigoid: clinical findings, immunopathogenesis, and significant associations. Medicine (Baltimore) 2003 May;82(3):177-86
Date
06/07/2003Pubmed ID
12792304DOI
10.1097/01.md.0000076003.64510.00Scopus ID
2-s2.0-0038247920 (requires institutional sign-in at Scopus site) 102 CitationsAbstract
We report the clinical and immunopathologic findings in a cohort of 35 patients with anti-epiligrin cicatricial pemphigoid (AECP). These patients have a mucosal predominant subepithelial blistering disease that is clinically indistinguishable from other forms of cicatricial pemphigoid. The mucosal surfaces of the mouth and eye are most commonly involved. The skin is also involved in most patients, but usually this is less severe than mucosal involvement. AECP is characterized by the binding of circulating IgG autoantibodies to the dermal side of 1M NaCl split human skin on indirect immunofluorescence microscopy. These IgG antibasement membrane autoantibodies target laminin 5, a heterotrimeric protein consisting of alpha3, beta3, and gamma2 subunits. IgG autoantibodies predominantly target the G domain within the alpha subunit. The presence of circulating IgG autoantibodies are specific for the diagnosis of AECP and are not seen in patients with other autoimmune blistering diseases or normal volunteers. Furthermore, we expand on data previously reported on the finding of an increased relative risk for solid cancer in patients with AECP, especially in the first year after blister onset. The majority of cancers documented in a cohort of 35 patients assembled over 12 years of study were adenocarcinomas that were at an advanced stage at their time of detection. This circumstance is thought to account for a high incidence of mortality among AECP patients who develop an associated cancer. AECP patients also demonstrate a significant risk for mortality as a consequence of treatment with systemic immunosuppressives. The current longitudinal study suggests that only a minority of AECP patients go into remission.
Author List
Egan CA, Lazarova Z, Darling TN, Yee C, Yancey KBMESH terms used to index this publication - Major topics in bold
AdultAged
Aged, 80 and over
Autoantibodies
Biopsy
Cell Adhesion Molecules
Cohort Studies
Female
Fluorescent Antibody Technique, Indirect
Humans
Immunoblotting
Immunoglobulin G
Immunohistochemistry
Laminin
Male
Middle Aged
Neoplasms
Pemphigoid, Benign Mucous Membrane
Precipitin Tests
Skin
