SG09 Vulvovaginal involvement and sexual dysfunction in women with ichthyosis: a cross-sectional questionnaire study

Abstract

Vulvovaginal involvement in ichthyosis is poorly characterized, despite well-recognized cutaneous morbidity in this group of rare epidermal differentiation disorders. We conducted a single-­centre, cross-sectional questionnaire study of adult women with genetically and clinically confirmed ichthyosis attending a tertiary dermatology service, to assess vulvovaginal symptoms, vulvar disease-specific quality of life and sexual function. Ten women (mean age 37.2 years, range 20–70) were enrolled, with ichthyoses including Netherton syndrome, epidermolytic ichthyosis, congenital ichthyosiform erythroderma, erythrokeratoderma variabilis and lamellar ichthyosis. Standardized pro formas were used to capture symptoms, gynaecological history and focused vulval examination, alongside three validated patient-reported outcome measures: Dermatology Life Quality Index (DLQI), Vulvar Quality of Life Index (VQLI) and Female Sexual Function Index (FSFI). Vulvovaginal symptoms were frequent: dryness (6 of 10), white discharge (5 of 10), itch (4 of 10), pain (2 of 10) and recurrent infections (2 of 10). Examination in five women showed lichenification, hyperkeratosis, superficial erosions, fine scaling and erythema, resembling chronic inflammatory vulvar dermatoses. Quality of life was markedly impaired, with a mean DLQI of 16.3, and 7 of 10 participants reporting a very large or extremely large effect on daily life. VQLI scores showed heterogeneous but often substantial vulvar impact (mean 15.1), with three women in the severe band. Sexual function was significantly reduced: five of the six sexually active women completing the FSFI scored below the 26-point threshold for dysfunction (mean 18.6), with deficits across desire, arousal, lubrication, orgasm, satisfaction and pain. This study highlights a high burden of vulvovaginal symptoms, lichen sclerosus-like vulval changes, profound quality-of-life impairment and sexual dysfunction in this small cohort of patients with ichthyosis. Routine enquiry about genital symptoms, targeted emollient-based care, multidisciplinary sexual and psychological support, and optimization of preventive gynaecological care should be incorporated in the assessment of patients with ichthyosis. Larger studies are needed to confirm these findings and inform evidence-based management pathways.