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Case Report/Case Series |

Three Cases of Linear IgA/IgG Bullous Dermatosis Showing IgA and IgG Reactivity With Multiple Antigens, Particularly Laminin-332

Masanobu Sakaguchi, MD, PhD1; Toshinori Bito, MD, PhD1; Yoshiko Oda, MD1; Ayuko Kikusawa, MD1; Chikako Nishigori, MD, PhD1; Takichi Munetsugu, MD, PhD2; Hiroo Yokozeki, MD, PhD2; Yuri Itotani, MD3; Toshiyuki Niguma, MD, PhD3; Daisuke Tsuruta, MD, PhD4; Chiharu Tateishi, MD, PhD4; Norito Ishii, MD5; Hiroshi Koga, MD, PhD5; Takashi Hashimoto, MD5
[+] Author Affiliations
1Division of Dermatology, Department of Internal Medicine Related, Faculty of Medicine, Kobe University Graduate School of Medicine, Hyogo, Japan
2Department of Dermatology, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan
3Department of Dermatology, National Hospital Organization, Higashihiroshima Medical Center, Higashihiroshima, Japan
4Department of Dermatology, Osaka City University Graduate School of Medicine, Osaka, Japan
5Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Fukuoka, Japan
JAMA Dermatol. 2013;149(11):1308-1313. doi:10.1001/jamadermatol.2013.5691.
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Importance  Linear IgA/IgG bullous dermatosis (LAGBD) is a relatively rare autoimmune bullous disease characterized by both IgA and IgG antibodies to epidermal basement membrane zone. The heterogeneity and pathogenesis of the LAGBD autoantigens have not been fully elucidated.

Observations  We report 3 Japanese cases of LAGBD (ages 81, 88, and 64 years; 1 woman and 2 men). The patients showed bullous and erosive lesions on the trunk and extremities with minimal mucosal lesions. Histopathological analysis revealed a subepidermal blister with neutrophilic infiltration with eosinophils in 2 cases. Direct and indirect immunofluorescence studies disclosed IgG and IgA antibasement membrane zone antibodies. In immunoblot analyses of various antigen sources, all cases showed IgG and IgA antibodies to various subunits of laminin-332, in addition to IgG and IgA reactivity with type VII collagen, laminin-γ1, and BP230 and BP180 recombinant proteins.

Conclusions and Relevance  Our studies revealed that the 3 LAGBD cases showed prominent IgG and IgA reactivity with laminin-332, which was only rarely reported. In addition, all cases showed IgG and IgA reactivity with other multiple antigens, indicating the role of epitope-spreading mechanisms initiated from laminin-332. The significance of IgA antibodies to laminin-332 should be studied in larger cohorts of both LAGBD and linear IgA bullous dermatosis.

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Figure 1.
Case 1

A and B, Clinical features. C, Histopathological features (hematoxylin-eosin, original magnification ×100). D and E, immunofluorescence (IF) features (D, direct IF for IgA [original magnification ×100]; and E, indirect IF of 1M sodium chloride–split skin section for IgA antibodies in the serum sample [original magnification ×40]). F-I, Immunoblot analyses (the results are shown in lanes labeled as “Patient” for both IgG and IgA antibodies). F, In purified human laminin-332, IgG antibodies in the control anti–laminin-332–type mucous membrane pemphigoid (MMP) serum sample reacted strongly with the 165-kDa and 145-kDa forms of laminin-α3, 140-kDa laminin-β3, and 105-kDa laminin-γ2 (lane 1), while IgG antibodies in the normal control serum sample showed no reactivity (lane 2). G, In normal human dermal extracts, IgG antibodies in the control epidermolysis bullosa acquisita (EBA) serum sample reacted with 290-kDa type VII collagen (lane 1), and IgG antibodies in the control anti–laminin-γ1 pemphigoid (p200) serum sample reacted with 200-kDa laminin-γ1 (lane 2). H, Immunoblot analysis using SuperSignal West Dura Chemiluminescent Substrate showed similar results. I, IgG antibodies in the control anti-BP180–type MMP serum sample (lane 1), but not in the normal control serum sample (lane 2), reacted with recombinant protein (RP) of the C-terminal domain.

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Figure 2.
Case 2

A and B, Clinical features. C, Histopathological features (hematoxylin-eosin, original magnification ×100). D and E, Immunofluorescence (IF) features (direct IF for IgG [D] and IgA [E] [original magnification ×100]). F-H, Immunoblot analyses (the results are shown in lanes labeled as “Patient” for both IgG and IgA antibodies), using purified human laminin-332 (F), normal human epidermal extracts (G), and recombinant protein (RP) of the C-terminal domain of BP180 (H).

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Figure 3.
Case 3

A and B, Clinical features. C, Histopathological features (hematoxylin-eosin, original magnification ×100). D and E, Immunofluorescence (IF) features (indirect IF of 1M sodium chloride–split skin sections for IgG [D] and IgA [E] antibodies [original magnification ×40]). F and G, Immunoblot analyses (the results are shown in lanes labeled as “Patient” for both IgG and IgA antibodies), using purified human laminin-332 (F), and recombinant protein (RP) of the NC16a domain of BP180 (G).

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