Pemphigus vulgaris (PV) is a potentially devastating autoimmune bullous disease that affects the mucosa and skin. It is characteristically associated with intra- epidermal blistering and cell–cell dissociation due to the presence of autoantibodies targeting desmoglein (dsg)

© 2013 The Authors. doi: 10.2340/00015555-1388 Journal Compilation © 2013 Acta Dermato-Venereologica. ISSN 0001-5555 Pemphigus vulgaris (PV) is a potentially devastating autoimmune bullous disease that affects the mucosa and skin. It is characteristically associated with intraepidermal blistering and cell–cell dissociation due to the presence of autoantibodies targeting desmoglein (dsg) 3 and dsg 1, i.e. transmembrane adhesion molecules of the desmosomal cadherin protein family (1–3). In most patients, PV characteristically starts with and affects the oral mucosa, although involvement of other mucosae may also occur (4–10). We have recently observed 2 patients, in whom the first clinical manifestation of PV consisted of chronic painful penile erosions, which represented a diagnostic challenge.