VULVAR HISTOPATHOLOGICAL AND IMMUNOHISTOCHEMICAL CHANGES IN PATIENTS WITH PRIMARY SJÖGREN SYNDROME

Background: Primary Sjogren Syndrome (pSS) is an autoimmune disease mostly affecting women, characterized by a lymphocyte-mediated infiltration and destruction of several exocrine glands, which causes mucosal dryness. Genital involvement is frequent and characterized by vulvar and vaginal dryness, dyspareunia and pruritus, that significantly impairs sexual function. However, despite the high frequency of genital involvement, few data were published about the histopathology of external genitalia in pSS. The studies performed until now show that vaginal and vulvar dryness are due to the presence of a vulvar inflammatory infiltrate and to the atrophy of minor and major vestibular glands, whose secretions are important for the sexual function. Objectives: To evaluate the presence and the characteristics of histopathological and immunohistochemical changes in vulvar tissues in women with pSS. Methods: Women with pSS (21 patients) underwent vulvar biopsies that have been evaluated for histopathological and immunohistochemical changes and finally compared with those obtained from 26 patients with lichen sclerosus. Results: An inflammatory infiltrate (composed predominantly by T lymphocytes (CD3+), sparse CD20+ B cells and mean CD4:CD8 T-cell ratio of 1.5) was evidenced in all 21 biopsies and classified in mild (10), moderate (11) and severe (0). No correlation was shown between vulvar inflammatory infiltrate score and salivary Chisholm e Mason score. No differences were found neither in gynecological symptoms neither 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.