The goal of this study was to evaluate the characteristics and the prevalence of histopathologic and immunohistochemical changes in vulvar biopsies, in women with Primary Sjögren Syndrome (pSS) and non-Sjögren Sicca Syndrome (nS-SS). Twenty-one women with pSS and 11 with nS-SS (investigated by xerophtalmia and xerostomia tests, biopsy of minor salivary glands, gynecological history, and gynecologic symptoms score) underwent vulvar biopsies, evaluated for histopathologic and immunohistochemicalchanges, and compared with those obtained from 26 patients with lichen sclerosus. An inflammatory infiltrate was present in 31/32 biopsies (96.9%); biopsies from pSS patients showed a mild infiltrate in 10 cases and a moderate infiltrate in 11 cases; and patients with nS-SS had a mild infiltrate in 8 biopsies and moderate infiltrate in the other 2 biopsies. By immunohistochemistry, the infiltrate was composed predominantly of T lymphocytes (CD3+), CD20+ B cells were sparse and mean CD4:CD8 T-cell ratio was 1.5. No differences were observed between the grading of the inflammatory infiltrate in nS-SS and pSS; no correlation was shown between vulvar inflammatory infiltrate score (mild or moderate) and salivary glands inflammatory score. No differences were found in gynecologic symptoms, as well as in clinical and demographical characteristics between patients with mild and those with moderate vulvar inflammatory score. A higher prevalence of moderate inflammatory infiltrate was observed in biopsies from women with lichen sclerosus than in pSS and nS-SS patients (61.5% vs. 27.5%, P=0.02). Women with pSS and nS-SS show a high and similar prevalence of vulvar inflammatory infiltrate. A gynecologic evaluation is needed both in pSS and nS-SS to assess genital involvement and, eventually, to address a therapy targeted to genital symptoms.