Mixed immunobullous disorder most consistent with the IgA-form of epidermolysis bullosa acquisita.
We describe a case of non-scarring, generalized, cutaneous and mucosal subepidermal bullous dermatosis that is characterized histopathologically by a neutrophilic infiltrate and strong linear staining with both IgA and IgG along the basement-membrane zone. Autoantibodies to collagen VII of both the IgA and IgG4 subtypes were detected by indirect immunofluorescence test, which led led to a diagnosis of epidermolysis bullosa aquisita (EBA). EBA is a subepidermal bullous disorder that is mediated by autoantibodies, which are directed against type VII collagen. The distinct clinical presentations of EBA are reviewed and discussed in the context of the unique autoantibody profile of this case.
Rizzo
Carina
C
Department of Dermatology, New York University, USA.
Votava
Henry J
HJ
Meehan
Shane A
SA
Kundu
Roopal
R
Franks
Andrew G
AG
D000328:Adult; D016107:Epidermolysis Bullosa Acquisita / Q000276:immunology* / Q000473:pathology; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology*
D007070:Immunoglobulin A