A 54-year-old woman comes to the office due to increasing vulvar pruritus and burning that is preventing sleep. The symptoms began about a year ago and have become more severe. In addition to the constant pruritus, the patient has had increasingly painful defecation. Two days ago, her vulva bled after intense scratching. She attributes her symptoms to dry skin and has been applying skin moisturizer daily. The patient is not sexually active. Medical history includes alopecia areata and symptomatic hot flashes that have occurred since menopause at age 51. Examination shows bilateral labia majora with excoriated, pale, thin skin. The labia minora are not visible, and there is severe narrowing of the introitus. The perianal skin is pale white, appears wrinkled, and has a small anal fissure. Which of the following is the most likely diagnosis in this patient?
Vulvar lichen sclerosus | |
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This patient has vulvar lichen sclerosus, a chronic, benign inflammatory disease common in postmenopausal women, particularly those with associated autoimmune disease (eg, alopecia areata). In early stages of the disease, the thin, wrinkled vulvar skin results in hypopigmented areas and increased skin sensitivity that causes an intense pruritus with excoriations that are commonly associated with the disease. Patients with lichen sclerosus often have perianal skin involvement in a figure-eight pattern that can result in painful defecation and anal fissures. In severe cases, normal anatomic structures may be obliterated or atrophied, such as loss of the labia minora and clitoral hood, which can cause narrowing of the vaginal introitus and dyspareunia.
Lichen sclerosus is diagnosed clinically; however, vulvar biopsy is recommended due to the association of lichen sclerosus with vulvar cancer. Treatment is with high-potency topical corticosteroids, which decreases inflammation, resolves symptoms, and reduces the risk of malignancy. In those in whom initial biopsy is deferred, biopsy is required if the lichen sclerosus is refractory to treatment.
(Choice A) Atopic dermatitis (ie, eczema) can present with intense pruritus. In contrast to this patient, those with atopic dermatitis typically have erythematous papules and vesicles. In addition, new-onset atopic dermatitis typically occurs in children age <5.
(Choice B) Vulvar lichen planus is an autoimmune condition that typically presents with pruritic, purple plaques that are sometimes associated with thin, white striae around the labia and vulva (ie, Wickham striae).
(Choice D) Lichen simplex chronicus occurs secondary to repetitive scratching. Patients develop a hyperplastic response that causes thickened, leathery skin, which is not seen in this patient.
(Choice E) A pinworm infection, although more common in children, can occur in adults and presents as intense nocturnal perianal pruritus. Patients may have excoriations but no other associated lesions.
(Choice F) Vulvovaginal atrophy (ie, genitourinary syndrome of menopause) can present with vulvar pruritus, thinned vulvar skin, and fusion of the labia minora that causes narrowing of the vaginal introitus. Vulvovaginal atrophy does not commonly involve the perianal region, making this diagnosis less likely.
Educational objective:
Vulvar lichen sclerosus is a chronic inflammatory disorder that causes intense vulvar pruritus. Examination findings include thin, wrinkled vulvar skin, often with perianal involvement. Treatment is with high-potency topical corticosteroids.