Xiaoguang Li1, Atsunari Tsuchisaka1, Hua Qian1, Kwesi Teye1, Norito Ishii1, Ryosuke Sogame1, Kazutoshi Harada2, Daiki Nakagomi2, Shinji Shimada2, Chiharu Tateishi3, Yoshiaki Hirako4, Takashi Hashimoto1. 1. Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, 67 Asahimachi, Kurume, Fukuoka 830-0011, Japan. 2. Department of Dermatology, Faculty of Medicine, University of Yamanashi, Chuo, Yamanashi, Japan. 3. Department of Dermatology, Osaka City University Graduate School of Medicine, Osaka, Japan. 4. Division of Biological Science, Graduate School of Science, Nagoya University, Nagoya, Aichi, Japan.
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.
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.
Entities:
Keywords:
integrin α6β4; laminin-332; laminin-γ1; linear IgA/IgG bullous dermatosis