Case of pemphigus with immunoglobulin G and A antibodies, binding to both the intercellular spaces and basement membrane zone

Abstract

We report a case involving a 62-year-old woman with in vivo-bound immunoglobulin (Ig)G and IgA antibodies in both the intercellular space (ICS) and basement membrane zone (BMZ). Her clinical and histopathological features were identical with those of pemphigus vulgaris, while the immunopathological findings suggested IgG/IgA pemphigus. Direct immunofluorescence (IF) showed in vivo-bound IgG and IgA antibodies in the ICS and BMZ, whereas indirect IF showed circulating IgG but not IgA antibodies in the ICS and BMZ. The anti-ICS IgG bound to desmoglein-3, while the anti-BMZ antibodies bound to the epidermal side of 1 mol/L NaCl-split skin. To the best of our knowledge, only two similar cases have been reported so far. Furthermore, we also examined IgG subclass distribution of the in vivo-bound and circulating anti-ICS and BMZ antibodies, and found that IgG1, IgG2 and IgG4 bound to ICS of the lesional skins, while IgG1 and IgG3 bound to the BMZ. The circulating anti-ICS antibodies belonged to IgG1 and IgG4, while the circulating anti-BMZ antibodies to IgG1, IgG2 and IgG4. We report a case involving a 62-year-old woman with in vivo-bound immunoglobulin (Ig)G and IgA antibodies in both the intercellular space (ICS) and basement membrane zone (BMZ). Her clinical and histopathological features were identical with those of pemphigus vulgaris, while the immunopathological findings suggested IgG/IgA pemphigus. Direct immunofluorescence (IF) showed in vivo-bound IgG and IgA antibodies in the ICS and BMZ, whereas indirect IF showed circulating IgG but not IgA antibodies in the ICS and BMZ. The anti-ICS IgG bound to desmoglein-3, while the anti-BMZ antibodies bound to the epidermal side of 1 mol/L NaCl-split skin. To the best of our knowledge, only two similar cases have been reported so far. Furthermore, we also examined IgG subclass distribution of the in vivo-bound and circulating anti-ICS and BMZ antibodies, and found that IgG1, IgG2 and IgG4 bound to ICS of the lesional skins, while IgG1 and IgG3 bound to the BMZ. The circulating anti-ICS antibodies belonged to IgG1 and IgG4, while the circulating anti-BMZ antibodies to IgG1, IgG2 and IgG4.

Case of bullous pemphigoid coexisting with anti‐desmoglein autoantibodies

“…Interestingly, apart from the BP cases shown in Table , cases of pemphigoid vegetans, pemphigoid gestationis and mucous member pemphigoid were also reported as having pemphigus autoantibodies. In addition, some cases of pemphigus were reported to have anti‐DEJ antibodies. In most of these cases, diagnosis was made according to their histological findings.…”

IgG/IgA-pemphigus — extremely rare blistering autoimmune diseases

“…Преимущественно нейтрофильный характер инфильтрата в коже больных IgG/ IgA-пузырчаткой обусловлен наличием на моноцитах и гранулоцитах специфических связывающих участков для IgA-Fc-рецептора [31]. В ряде случаев в очагах поражения кожи у больных IgG/IgA-пузырчаткой в составе инфильтрата преобладали лимфоциты [32]. Отмечалось также присутствие в инфильтрате эозинофилов [15,17].…”

Antigen recognition in the pathogenesis of immunoglobulin A-related autoimmune bullous diseases

“…The most common target antigen detected by ELISA in the sera of the IgG/IgA pemphigus patients is DSG1. Anti-DSG3, anti-desmocollin, and anti-desmoplakin antibodies were also detected in the sera of some patients [ 11 ]. Despite these findings, the pathogenesis of IgG/IgA pemphigus and whether IgA or IgG antibody causes blisters in this disease remain unknown.…”