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.
Keywords: integrin α6β4; laminin-332; laminin-γ1; linear IgA/IgG bullous dermatosis.