A case of IgG/IgA pemphigus presenting malar rash-like erythema.

Journal Compilation © 2012 Acta Dermato-Venereologica. ISSN 0001-5555 Pemphigus is an autoimmune mucocutaneous bullous disease characterized by auto-antibodies against cell surface antigens of epidermal keratinocytes. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are the major subtypes. Several other variants have been proposed, including pemphigus erythematosus, pemphigus vegetans, pemphigus herpetiformis (PH), and paraneoplastic pemphigus. Deposition of IgG on epidermal keratinocyte cell surfaces and circulating anti-cell surface antibodies are characteristic in pemphigus. Cases involving IgA deposition on epidermal keratinocyte cell surfaces have been reported as IgA pemphigus. IgA pemphigus is divided into 4 subgroups based on clinical manifestation: subcorneal pustular dermatosis type, intraepidermal neutrophilic IgA dermatosis type, pemphigus foliaceus type, and pemphigus vulgaris type. Cases involving deposition of both IgG and IgA on keratinocyte cell surfaces have been reported (1–13). Some authors describe them as IgG/IgA pemphigus (1). Seventeen such cases have been reported so far, and heterogeneity of clinical features and target antigen has been detected in this group of pemphigus.