According to the extent of affected skin, three types of PV can be distinguished: (i) the mucosal-dominant type with limited cutaneous involvement (Dsg 3-autoantibodies are predominant), (ii) the mucocutaneous type with both mucosal and cutaneous involvement (Dsg3- and Dsg1-autoantibodies are equally predominant) and the cutaneous type with predominant anti-Dsg1 and pathogenically weak anti-Dsg3 autoantibodies (1). Here, DSG1 is linked to acquired polycythemia vera.