Excerpt from Lichen Sclerosus et Atrophicus

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Background

Lichen sclerosus (LS) is a chronic inflammatory dermatosis that results in white plaques with epidermal atrophy. LS has both genital and extragenital presentations. It is reported under a variety of other appellations such as lichen sclerosus et atrophicus (dermatological literature), balanitis xerotica obliterans (glans penis presentation), and kraurosis vulvae (older description of vulvar presentation). An increased risk of squamous cell carcinoma may exist in vulvar disease, but the precise increase in risk and what cofactors (human papilloma virus infection or prior radiotherapy) may be involved are not yet completely defined. In large series, genital presentations, both vulvar and penile, outnumber extragenital reports by more than 5:1.

Pathophysiology

Inflammation and altered fibroblast function in the papillary dermis leads to fibrosis of the upper dermis. Genital skin and mucosa are affected most frequently, but extragenital LS does occur, and even rare oral presentations are reported. Several studies have recently identified the presence of autoantibodies to the glycoprotein extracellular matrix protein 1 (ECM1). This may be associated with histologic evidence of vasculitis in some cases and may lead to reduplication of the basement membrane in blood vessel walls. The exact role of these antibodies and the changes seen in the microvasculature are currently under investigation. Systemic disease or involvement of other organ systems, unlike scleroderma, is not described, although many more authors are describing LS and scleroderma as closely related entities; many cases of coexistent LS/scleroderma have been reported.

Frequency

International

The population rate is unknown. Male genital LS is seen almost exclusively in uncircumcised men and boys. The rate of circumcision in a given population would thus influence the rate in this subset.

Mortality/Morbidity

LS has no associated increased mortality unless the patient develops a malignancy in the area. Cancer arising in extragenital presentations is described only rarely and may be coincident with other factors. Many pediatric cases will improve with puberty. Extragenital cases and many genital cases are asymptomatic except for the cosmetic aspect or pruritus. Recalcitrant cases, especially those associated with erosion or progressive scarring, may result in severe sexual dysfunction.

Race

LS, both genital and extragenital, has no known racial predilection.

Sex

The male-to-female ratio is 1:6, with female genital cases making up the bulk of reports.

Age

Up to 15% of cases are in children with the majority being vulvar presentations. A study of foreskins submitted after therapeutic circumcision for phimosis revealed many cases of unrecognized LS. Extragenital LS is rare in children.

Please click here to view the full topic text: Lichen Sclerosus et Atrophicus