Localized Linear IgA/IgG Bullous Dermatosis

Abstract

Linear IgA/IgG bullous dermatosis (LAGBD) is an auto-immune blistering disease characterized by the local accumulation of IgA- and IgG-class anti-basement membrane autoantibodies. It typically presents as a generalized pruritic vesiculobullous eruption. No cases of localized LAGBD have yet been reported. We report a case of a 78-year-old man with LAGBD localized to the perianal area. The patient complained of suffering from persistent ulcers around the anus for more than 3 years. Physical examination revealed several blisters and ulcers up to 2-cm in diameter around the anus. No lesions were found elsewhere on the body. Histological analysis of a skin biopsy revealed subepidermal blistering, while direct immunofluorescence showed the linear deposition of IgA and IgG antibodies at the dermoepidermal junction. Indirect immunofluorescence of normal human skin whose layers had been separated using 1M NaCl showed the binding of both IgA and IgG to the epidermal side. Immunoblotting demonstrated the presence of circulating IgA and IgG autoantibodies that bound to a 120-kDa protein. This is the first case of localized LAGBD whose skin lesions were restricted to the perianal region.

Linear IgA Bullous Dermatosis: A Retrospective Study of 23 Patients in Denmark

“…Hence, the diagnosis in the latter case was established based on the clinical characteristics, the lack of symptoms from the intestines, and a negative screening for coeliac disease. Mixed deposits are previously described in a number of cases (11)(12)(13)(14)(15)(16)(17)(18). Their significance remains uncertain but it may be confusing and make the differential diagnosis more difficult.…”

Linear immunoglobulin A/G bullous dermatosis associated with ulcerative colitis

“…Recently, immunoblot studies have indicated that LAGBD sera show circulating IgA and IgG antibodies reactive with various autoantigens: BP230, BP180 NC16a domain, 97/120‐kDa LAD‐1, BP180 C‐terminal domains, laminin‐332, 290‐kDa type‐VII collagen and 200‐kDa laminin‐γ1 . Our patient showed that IgG antibodies reacted with the BP180 NC16a domain and 120‐kDa LAD‐1 in immunoblotting analyses, and IgG antibodies to BP230 in ELISA.…”

“…Patients with LAGBD have responded well to DDS therapy in the same manner as that for LABD treatment . DDS has efficacy against skin diseases characterized by neutrophil‐rich infiltrates.…”

Pemphigoid Nodularis with Diverse IgG, IgA and IgE Antibodies Showing Neutrophilic Papillary Microabscesses

“…Considering that DIF and IIF showed IgA anti-BMZ antibodies, our patient can be diagnosed with linear IgA/IgG bullous dermatosis (LAGBD), or a variant of linear IgA bullous dermatosis (LABD) (2). Interestingly, immunoblotting analysis, IgG, but not IgA, antibodies was shown to react with LAD-1, the shedded ectodomain of BP180.…”