An 11-year-old girl is brought to the office for vulvar pruritus that has increased over the past 3 months and now causes her to awaken from sleep. She tried applying her sister's diaper cream but had no relief of symptoms. The patient has also developed perianal pruritus and constipation. She has had no vaginal or rectal bleeding and no abnormal vaginal discharge. The patient has allergic rhinitis managed with a glucocorticoid nasal spray. She has had no surgeries. Vaccinations are up to date. The patient has not reached menarche. She has no known drug allergies. Weight and height are at the 50th and 45th percentile, respectively. Blood pressure is 98/62 mm Hg and pulse is 84/min. Physical examination reveals Tanner stage 2 breast and pubic hair development. Thin white lesions cover the vulva and extend over the perineum and around the anus. The labia majora and minora are edematous and have areas of thickened skin. Multiple excoriations appear throughout the vulva. There is no evidence of trauma. Which of the following is the most likely diagnosis in this patient?
Vulvar lichen sclerosus | |
Epidemiology |
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Vulvar lichen sclerosus is a benign, chronic inflammatory condition that commonly causes thinning of the vulvar skin in hypoestrogenic populations (eg, prepubertal girls, postmenopausal women). In lichen sclerosus, white atrophic papules form and eventually merge into plaques, leading to thin white lesions over the vulva. Chronic inflammation results in vulvar and perianal pruritus, at times so severe that it awakens patients from sleep. Excessive scratching can result in excoriations, lichenification (ie, thickened skin), and edema of the labia. Lichenification of the perianal area region can result in anal fissures and constipation.
Although biopsy is recommended in adults to exclude an underlying malignancy (eg, vulvar cancer), children have no associated malignancy risk and can be diagnosed clinically. First-line treatment is the same for adults and children: superpotent topical corticosteroids.
(Choice A) Atopic dermatitis can present with an erythematous, edematous vulva in patients with a history of allergic rhinitis. However, symptoms commonly respond to diaper cream.
(Choice B) Labial adhesions are common in infants and girls age <2. Patients may have vulvar pruritus; however, this is typically secondary to fusion of the labia at the midline, which causes a pinpoint vaginal opening and difficulty voiding, a finding not seen in this patient. In addition, labial adhesions are not associated with lichenification and have no perianal involvement.
(Choice D) Pinworm infections can cause perianal pruritus, which is more pronounced at night. However, there are no associated vulvar lesions.
(Choice E) Vulvovaginal candidiasis is uncommon in prepubertal girls with no recent antibiotic therapy or immunosuppression. It typically presents with a thick, clumpy vaginal discharge.
Educational objective:
Vulvar lichen sclerosus commonly presents in prepubertal girls with pruritus and thin, white lesions of the vulva and perianal region. Treatment is with superpotent topical corticosteroids.