CSF2 and pachyonychia congenita: Regarding our view that the CD86+ plasmacytoid DC% DC immune phenotype is a risk factor for PC, some studies have pointed out that in the DC of PC, DC is an important participant in the dysfunction of its immunosuppressive microenvironment.[63–68] In a randomized controlled trial administering different dosages of recombinant human granulocyte/macrophage colony-stimulating factor (rhGM-CSF) to patients with biochemically relapsed PC, those in group B (receiving 250 μg/m2 thrice weekly) had reduced plasmacytoid DC counts,[69] partially explaining our identified risk factor.