Abstract
Background
Since the original description by Zone et al in 1994, the disease entity and target antigens in linear IgA/IgG bullous dermatosis (LAGBD) have not been clarified in 20 years.
Objectives
To determine autoantibodies and autoantigens in a new LAGBD case which showed atypical clinical and histopathological findings without apparent mucosal involvement.
Materials and Methods
We performed various indirect immunofluorescence and immunoblotting studies.
Results
Indirect immunofluorescence of 1M NaCl-split skin showed IgG and IgA reactivity with both epidermal and dermal sides. Immunoblotting studies using various antigen sources revealed circulating IgG and IgA antibodies reactive with laminin-332, laminin-γ1 and integrin α6β4 in various patterns. Absorption study using recombinant proteins of laminin-γ1 indicated that the patient serum reacted with different epitopes between laminin-γ1 and laminin-γ2.
Conclusions
This study presented for the first time a LAGBD patient with IgG and IgA antibodies to various laminins and integrins.
Similar content being viewed by others
References
Hashimoto T, Ishii N, Ohata C, et al. Pathogenesis of epidermolysis bullosa acquisita, an autoimmune subepidermal bullous disease. J Pathol 2012; 228: 1–7.
Sugi T, Hashimoto T, Hibi T, et al. Production of human monoclonal anti-basement membrane zone (BMZ) antibodies from a patient with bullous pemphigoid (BP) by Epstein-Barr virus transformation. Analyses of the heterogeneity of anti-BMZ antibodies in BP sera using them. J Clin Invest 1989; 84: 1050–5.
Matsumura K, Amagai M, Nishikawa T, et al. The majority of bullous pemphigoid and herpes gestationis serum samples react with the NC16a domain of the 180-kDa bullous pemphigoid antigen. Arch Dermatol Res 1996; 288: 507–9.
Nie Z, Hashimoto T. IgA antibodies of cicatricial pemphigoid sera specifically react with C-terminus of BP180. J Invest Dermatol 1999; 112: 254–5.
Ishii N, Yoshida M, Hisamatsu Y, et al. Epidermolysis bullosa acquisita sera react with distinct epitopes on the NC1 and NC2 domains of type VII collagen: study using immunoblotting of domainspecific recombinant proteins and postembedding immunoelectron microscopy. Br J Dermatol 2004; 150: 843–51.
Dmochowski M, Hashimoto T, Bhogal BS, et al. Immunoblotting studies of linear IgA disease. J Dermatol Sci 1993; 6: 194–200.
Hisamatsu Y, Nishiyama T, Amano S, et al. Usefulness of immunoblotting using purified laminin 5 in the diagnosis of anti-laminin 5 cicatricial pemphigoid. J Dermatol Sci 2003; 33: 113–9.
Dainichi T, Kurono S, Ohyama B, et al. Anti-laminin gamma-1 pemphigoid. Proc Natl Acad Sci U S A 2009; 106: 2800–5.
Letko E, Bhol K, Foster SC, et al. Influence of intravenous immunoglobulin therapy on serum levels of anti-beta 4 antibodies in ocular cicatricial pemphigoid. A correlation with disease activity. A preliminary study. Curr Eye Res 2000; 21: 646–54.
Malik M, Gurcan HM, Christen W, et al. Relationship between cancer and oral pemphigoid patients with antibodies to alpha6-integrin. J Oral Pathol Med 2007; 36: 1–5.
Zone JJ, Pazderka Smith E, Powell D, et al. Antigenic specificity of antibodies from patients with linear basement membrane deposition of IgA. Dermatology 1994; 189(Suppl 1): 64–6.
Sakaguchi M, Bito T, Oda Y, et al. Three cases of linear IgA/IgG bullous dermatosis showing IgA and IgG reactivity with multiple antigens, particularly laminin-332. JAMA Dermatol 2013; 149: 1308–13.
Chan LS, Traczyk T, Taylor TB, et al. Linear IgA bullous dermatosis. Characterization of a subset of patients with concurrent IgA and IgG anti-basement membrane autoantibodies. Arch Dermatol 1995; 131: 1432–7.
Honoki K, Muramatsu T, Tsubakimoto A, et al. Linear IgA bullous dermatosis with circulating IgG autoantibodies to the 230 kD epidermal antigen. J Dermatol 1998; 25: 503–9.
Hertl M, Budinger L, Christophoridis S, et al. IgG and IgA antibodies in linear IgA/IgG bullous dermatosis target the ectodomain of bullous pemphigoid antigen 2. Br J Dermatol 1999; 140: 750–2.
Metz BJ, Ruggeri SY, Hsu S, et al. Linear IgA dermatosis with IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180): evidence for a distinct subtype. Int J Dermatol 2004; 43: 443. 6.
Shimizu S, Natsuga K, Shinkuma S, et al. Localized linear IgA/IgG bullous dermatosis. Acta Derm Venereol 2010; 90: 621–4.
Hirako Y, Usukura J, Uematsu J, et al. Cleavage of BP180, a 180-kDa bullous pemphigoid antigen, yields a 120-kDa collagenous extracellular polypeptide. J Biol Chem 1998; 273: 9711–7.
Ishii N, Ohyama B, Yamaguchi Z, et al. IgA autoantibodies against the NC16a domain of BP180 but not 120-kDa LAD-1 detected in a patient with linear IgA disease. Br J Dermatol 2008; 158: 1151–3.
Miyamoto S, Chikazu D, Yasuda T, et al. A case of oral mucous membrane pemphigoid with IgG antibodies to integrin alpha6beta4. Br J Dermatol 2014; 171: 1555–7.
Li X, Qian H, Takizawa M, et al. N-linked glycosylation on laminin gamma1 influences recognition of anti-laminin gamma1 pemphigoid autoantibodies. J Dermatol Sci 2015; 77: 125–9.
Author information
Authors and Affiliations
Corresponding author
Additional information
These three authors contributed equally to this study.
About this article
Cite this article
Li, X., Tsuchisaka, A., Qian, H. et al. Linear IgA/IgG bullous dermatosis reacts with multiple laminins and integrins. Eur J Dermatol 25, 418–423 (2015). https://doi.org/10.1684/ejd.2015.2555
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1684/ejd.2015.2555