Nevertheless, in some patients no link between autoantibody profile and non-stereotypical clinical manifestation was noted, that led to distinction of, the so called, atypical variant of PV (e.g., cutaneous PV[cPV] with anti-DSG3 or anti-DSG1/3 IgG autoantibodies) (10, 11). Here, DSG3 is linked to acquired polycythemia vera.