Abstract
Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition affecting the anogenital region, which may present in a prepubertal or adolescent patient. The most popular theories are its autoimmune and genetic conditioning, although theories concerning hormonal and infectious etiology have also been raised. The most common presenting symptoms of VLS is vulva pruritus, discomfort, dysuria and constipation. In physical examination, a classic “Figure 8” pattern is described, involving the labia minora, clitoral hood, and perianal region. The lesions initially are white, flat-topped papules, thin plaques, or commonly atrophic patches. Purpura is a hallmark feature of VLS. The treatment includes topical anti-inflammatory agents and long-term follow-up, as there is a high risk of recurrence and an increased risk of vulvar cancer in adult women with a history of lichen sclerosus. This article reviews vulvar lichen sclerosus in children and provides evidence-based medicine principles for treatment in the pediatric population. A systematic search of the literature shows recurrence of VLS in children. Maintenance regimens deserve further consideration.
Highlights
Health 2021, 18, 7153. https://Vulvar lichen sclerosus (VLS) is a chronic inflammatory disease of unclear etiology.The most popular theories are its autoimmune and genetic conditioning, theories concerning hormonal and infectious etiology have been raised [1]
Numerous case reports of twin, sibling and mother–daughter relationships contribute to a genetic factor in the etiology of lichen sclerosus et atrophicus [13]
In only 16% of cases, girls are diagnosed with vulvar lichen sclerosus in the initial stage of the disease
Summary
Vulvar lichen sclerosus (VLS) is a chronic inflammatory disease of unclear etiology. VLS manifests in lesions in vulvar mucosa, which often spreads to the skin of the anus [1]. The symptoms of this condition may include whitening of the perineal area, and itching, burning, discomfort, vaginal bleeding, and dysuria, which in sexually active girls may be mistaken for symptoms of urogenital infection [2]. There is good evidence for the use of high-potency corticosteroids as the initial treatment, but the maintenance treatment and most effective long-term management strategies are not established. Well-conducted randomized controlled trials, with long-term follow-up in the pediatric population are required in order to establish VLS treatment.
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