The expanding spectrum of I g A pemphigus: a case report and review of the literature

Summary I g A pemphigus ( IGAP ) is a rare, distinct variant of pemphigus, currently classified, depending upon the histological features, immunofluorescence staining pattern and autoantibody profile, into two types: subcorneal pustular dermatosis ( SPD ) and intraepidermal neutrophilic I g A dermatosis. In a patient with a widespread blistering disease of the skin resembling SPD ‐type IGAP , we demonstrate the coexistence of I g A reactivity to both epidermal (desmocollins 2 and 3) and basement membrane‐associated ( BP 180) proteins, suggesting the coexistence of atypical IGAP and linear I g A bullous dermatosis, respectively. This case, together with 20 previous reports of atypical IGAP , underscores the limitations of current classification schemes. Therefore, we suggest reclassifying these cases under the general term ‘ IGAP spectrum’.