Pediatric Vulvar Lichen Sclerosus—A Review of the Literature

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.

Comparison of lichen sclerosus in boys and girls: A systematic literature review of epidemiology, symptoms, genetic background, risk factors, treatment, and prognosis

“…Clinical signs are hypopigmentation, erythema, fissures, hyperpigmentation, atrophy, ecchymosis, and/or keratotic papules. Often a “figure‐of‐8” pattern is reported affecting the labia minora, clitoris, and perianal region 19,22 . Erosions and can be present, often due to scratching.…”

“…Often a "figure-of-8" pattern is reported affecting the labia minora, clitoris, and perianal region. 19,22 Erosions and can be present, often due to scratching. Vascular lesions such as angiokeratomas and telangiectasias are occasionally described and are usually asymptomatic.…”

“…Hypopigmentation, hyperpigmentation, erythema, fissures, atrophy, keratotic papules 19,22 Telangiectasias/purpura, angiokeratomas (rare) [23][24][25] Labial fusion 27,28 Hypopigmentation distal portion of the prepuce 35 Phimosis, scarring, secondary buried penis, balanitis Erythema, telangiectasias/purpura 11,35,36 Histopathology Hyperkeratosis (96%), basal vacuolization of keratinocytes (88%), lymphocytic exocytosis (91%), dermal sclerosis (99%) and epidermal atrophy (50%) 43 Hyperkeratosis (82.9%), hyalinization (100%), basal cell degeneration (56.1%), lymphocytic infiltration (100%), fibrin deposition (7.3%) 32 In 67% dermoepidermal clefts 44…”

Pediatric and Adolescent Gynecology — diagnostic and therapeutic trends

“…Another extremely important issue in pediatric gynecology are dermatoses of the vulva with special attention to lichen sclerosus vulgaris (VLS) -a chronic inflammatory disease of unclear etiology. VLS is characterized by clearly demarcated, pallid, atrophic skin lesions, involving lesions on the mucous membranes of the vulva and anal skin ("figure-of-eight" sign) [7,8]. 15-34% of cases among women and about 14% among girls coexist with allergies or autoimmune diseases, such as vitiligo, thyroiditis, type 1 diabetes, psoriasis, celiac disease and alopecia areata [9].…”

“…Early recognition and prompt implementation of treatment (0.05% clobetasol propionate ointment, 1 st -line therapy) is crucial in preventing long-term complications of lichen sclerosus vulgaris -especially in girls, such as adhesions, resorption of the labia minora, clitoris [7,8].…”