Lichen Sclerosus
Information has been excerpted from VisualDx clinical decision support system as a public health service. Additional information, including symptoms, diagnostic pearls, differential diagnosis, best tests, and management pearls, is available in VisualDx.
Synopsis
Lichen sclerosus (LS) is a chronic inflammatory and fibrosing dermatosis. Lesions originate as white or yellow papules and evolve to atrophic or white ivory patches, ecchymoses, and sclerosis. They occur mostly on the genitalia, perineum, or perianal skin but can appear anywhere. It is more common in females, with two peaks in age distribution: prepubertal children and postmenopausal women. Genetic and autoimmune factors are thought to contribute to the pathogenesis of LS.
Atrophic LS lesions of the penis are known as balanitis xerotica obliterans. The majority of cases of male genital LS occur in uncircumcised males.
In children, it is the most common skin condition mistaken for sexual abuse. Although onset is typically around 35-45 years of age, about 7%-15% of cases begin in childhood.
In males, LS may be asymptomatic, although it often results in pruritus and burning. Additionally, there may pain on defecation, constipation, dysuria, or anal or genital bleeding. A major goal of treatment for male pediatric anogenital LS is to prevent urethral stricture.
In females, LS commonly affects the vulvar and perianal skin, producing a “keyhole” area of hypopigmentation, often with scarring and loss of normal architecture. Lesions typically begin as white or yellow papules and can progress to white plaques with areas of atrophy, friability, and submucosal hemorrhage incited by only minimal trauma or irritation.
Misdiagnosis for sexual abuse most often occurs when the presentation is bullous, hemorrhagic, or erosive. The presence of hypopigmentation, atrophic skin, and telangiectasias should help differentiate this condition from sexual maltreatment. Findings of confirmed sexual abuse are primarily associated with acute injuries to the genitalia, anus or oral cavity, genital scarring, sexually transmitted diseases, and hymenal transection or evidence of a deep notch. However, case reports of LS coexisting with child abuse or resulting from trauma associated with repeated sexual maltreatment have been documented.
Childhood sexual abuse can affect children of all ages and economic and cultural backgrounds. Practitioners working with this population should consider this diagnosis routinely. Childhood sexual abuse is a serious allegation with major repercussions to a child and their family, and the differential diagnosis of genital lesions suggestive of sexual abuse includes LS and other anogenital dermatoses.
Genital LS confers a small risk of squamous cell carcinoma (SCC).
Look For
In females, sharply circumscribed, hypopigmented or depigmented plaques surround the clitoris and labia minora extending down the interlabial sulcus to the perianal area, sometimes referred to as a “figure-of-8” distribution. It does not involve the vaginal mucosa. The surface is atrophic and described as “cigarette paper like” or “cellophane like.” Repeated scratching results in secondary changes of excoriations, petechiae, erosions, purpura, ulcerations, and skin thickening. Scarring is seen with flattening of the normal arch, texture, and loss of the clitoris, labia minora, and narrowing of the introitus. However, minor loss of normal vulvar architecture and mild signs and symptoms may be all that heralds early vulvar LS.
Male genital lesions are often confined to the glans, urethra, and prepuce, but the perianal area may also be affected. Often, the presenting lesion is a sclerotic band or ring at the edge of the prepuce. LS may also be associated with phimosis.
Rare presentations include hemorrhagic bullae or purpura. Extragenital lesions occur in up to 10% of children, especially in areas of friction (neck, upper torso, buttocks, and inner thighs). These sites may show hypopigmentation and prominent follicles with hyperkeratotic plugging.
The full text and image collection is available to VisualDx subscribers.
Prepare for Everything
Get the only system designed for point-of-care visual diagnosis of common and rare medical disorders as well as emerging and re-emerging infectious diseases.
Your first 7 days are free. Cancel any time.