The aim of this study was to characterize retrospectively the clinical phenotype of patients with pSS with a non-identified ANA profile and no other concurrent autoimmune rheumatic diseases (ARD), in a comparison with that of patients with pSS with negative ANA, positive typical ANA (anti-Ro/SSA and/or La/SSB), and positive atypical ANA. The gene discussed is CALR; the disease is peeling skin syndrome.