Case of bullous pemphigoid coexisting with anti‐desmoglein autoantibodies

Abstract

A 79-year-old Japanese woman had clinical and histopathological features of bullous pemphigoid, while direct immunofluorescence test revealed C3 and immunoglobulin G depositions in the lower cell surfaces of the epidermis in addition to those in the dermoepidermal junction. Chemiluminescent enzyme immunoassays were positive for desmoglein-1 and -3 antibodies in addition to anti-BP180 antibodies. In an immunoblotting study, antibodies against both 180-kDa bullous pemphigoid antigen and 130-kDa pemphigus vulgaris antigen were detected. Based on these results, bullous pemphigoid coexisting with anti-desmoglein autoantibodies was diagnosed in this case.

Anti‐desmogleins autoantibodies detected by ELISA and blotting in bullous pemphigoid: what do they mean?

“…PF and PV are prevalent in southeastern Brazil, but BP and pemphigus association has not been observed in this region yet. Our results agree with previous reports revealing the presence of anti‐BP180 and anti‐BP230 concomitantly with anti‐DSG1 and anti‐DSG3 antibodies but with no clinical coexistence with PF or PV, suggesting that anti‐DSG are nonpathogenic antibodies . While DSG1 is expressed to a higher degree in the superficial epidermis, DSG3 is expressed in the lower portion of the epidermis and therefore close to basement membrane zone, where BP180 and BP230 are located.…”

“…On the other hand, pemphigus preferentially involves autoantibodies against desmogleins (DSG): pemphigus vulgaris (PV) against DSG3 and DSG1, and pemphigus foliaceus (PF) exclusively against DSG1 . Transitions from pemphigus to BP and vice versa have been reported . Unusually, BP patients have also shown autoantibodies against DSG with no clinical manifestation of pemphigus, and this event deserves attention .…”

“…Transitions from pemphigus to BP and vice versa have been reported . Unusually, BP patients have also shown autoantibodies against DSG with no clinical manifestation of pemphigus, and this event deserves attention . Fifty BP patients diagnosed at the University Hospital, Ribeirão Preto Medical School, University of São Paulo, southeastern Brazil, between 1993 and 2016, were tested for anti‐DSG1 and anti‐DSG3 antibodies using commercial ELISA kit (MBL, Nagoya, Japan).…”

Anti-desmoglein autoantibody in a patient with bullous pemphigoid - A case report

“…[ 1 ] Patient diagnosed with BP having autoantibody against desmoglein (Dsg) with no manifestation of pemphigus has been reported and transition between BP and PV and vice versa has also been reported. [ 2 ] We report a case of bullous pemphigoid coexisting with anti-desmoglein autoantibodies. A 60-year-old female presented with the history oral and genital ulcers followed by bulla over the chest, back and extremities since 4 months.…”

“…The presence of additional anti-Dsg-3 antibody suggest that it could be due to ES. [ 2 ]An epitope is an antigenic determinant, on the surface of an antigenic molecule, to which a single antibody binds. [ 3 ]The ES phenomenon is when tissue damage from a primary inflammatory process causes the release and exposure of a previously sequestered antigen, leading to a secondary autoimmune response against the newly released antigens.…”