The Immunoglobulin A Antibody Response in Clinical Subsets of Mucous Membrane Pemphigoid

Abstract

Background: Mucous membrane pemphigoid (MMP) is an immunobullous disease. In MMP there is frequently a mixed antibody response with the presence of IgA and/or IgG antibodies directed toward basement membrane zone antigens. The IgG antibody response in MMP has been studied, but the antigens to which the IgA antibodies react have not been studied. Objective: To determine the IgA autoantibody reactivity profiles in patients with MMP. Methods: Patients who had both ocular and oral MMP were compared with patients who had ocular or oral MMP and with patients who had cutaneous linear IgA disease (LABD) by Western immunoblot studies. Results: Five of 15 MMP patients and 1 of 5 LABD patients had IgA antibodies reactive with the 180-kD bullous pemphigoid antigen. Seven of 15 MMP patients had IgA antibodies reactive with the 97-kD LABD antigen. Conclusion: Major antigens in IgA MMP are the 180-kD bullous pemphigoid antigen and the 97-kD LABD antigen.

IgG, IgA and IgE autoantibodies against the ectodomain of BP180 in patients with bullous and cicatricial pemphigoid and linear IgA bullous dermatosis

“…28 Sera of BP patients with severe disease, including mucous membrane involvement, have recently been shown to contain both IgG and IgA reactive with components of the dermoepidermal BM. 29,30 However, Kirtschig and Wojnarowska did not find a direct correlation between IgA reactivity to 180-and 230-kDa BM proteins and the occurrence of mucosal lesions in BP. 31 The detection of BP180-reactive IgE autoantibodies in all BP sera studied is interesting in light of the identification of IgE reactive with BP230 in patients with severe BP.…”

“…This finding is in line with the recent identification of BP180‐reactive IgA in a patient with CP. 28 Sera of BP patients with severe disease, including mucous membrane involvement, have recently been shown to contain both IgG and IgA reactive with components of the dermoepidermal BM 29,30 . However, Kirtschig and Wojnarowska did not find a direct correlation between IgA reactivity to 180‐ and 230‐kDa BM proteins and the occurrence of mucosal lesions in BP.…”

Linear IgA disease and desquamative gingivitis: time for inclusion in mucous membrane pemphigoid

“…On the basis of the above mentioned arguments, we believe that the diagnosis of LAD can not be confirmed in any of the cases with exclusive mucosal involvement reported to date (including those by (Leao et al , 2008), but in those mentioning a positive and specific IB (Egan et al , 1999; Letko et al , 2000).…”

“…An exclusive mucosal involvement has been reported very rarely in LAD (Table 1) (Kumar et al , 1980; Porter et al , 1990, 1992; Kirtschig et al , 1998; Cohen et al , 1999; Egan et al , 1999; Lazzaro and Lazzaro, 1999; Letko et al , 2000; Eguia del Valle et al , 2003; Talhari et al , 2006; Angiero et al , 2007; Lewis et al , 2007). All 14 patients were adult (mean age 61 years, range 29–79); males / females ratio was 7/6.…”

Linear Immunoglobulin A Bullous Dermatosis: Need for an Agreement on Diagnostic Criteria

“…Moreover, to define predominance of IgA based on immunofluorescence staining intensity is very complicated, since the staining intensity critically depends on the experimental conditions, including dilution and type of the second step antibodies used for detection of the tissue-bound deposits. This question becomes more important considering that linear IgA deposits have been reported in several cases of subepidermal blistering diseases other than LABD, including BP [11] , mucous membrane pemphigoid (MMP) [12] , pemphigoid gestationis [13] and EBA [14] . Another important question about the immunopathological features of LABD concerns the presence of circulating IgA antibodies against BMZ antigens.…”