A subset of pSS patients with anti-centromere positivity develops a clinical phenotype overlapping between SS and systemic sclerosis with a higher age, more frequent Raynaud’s phenomenon and keratoconjonctivitis sicca and a lower proportion of anti-Ro/SSA and anti-La/SSB, rheumatoid factor, leukocytopenia and hypergammaglobulinemia [159,299]. Here, CALR is linked to peeling skin syndrome.