Lichen Sclerosus et Atrophicus

Updated: Apr 10, 2017
  • Author: Jeffrey Meffert, MD; Chief Editor: William D James, MD  more...
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Overview

Background

Lichen sclerosus (LS) is a chronic inflammatory dermatosis that results in white plaques with epidermal atrophy and scarring. Lichen sclerosus has both genital and extragenital presentations and also goes by the names 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 genital disease, but the precise increase in risk and what cofactors (human papillomavirus infection or prior radiotherapy) may be involved are not yet completely defined. In patients who have been treated for vulvar cancer, the presence or absence of lichen sclerosus does not appear to affect the timing of recurrence. In large series, genital presentations, both vulvar and penile, outnumber extragenital reports by more than 5:1.

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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 lichen sclerosus does occur, and even rare oral presentations are reported. The role that hypoxia and ischemia have in the initial cellular and vascular damage is supported by the finding of increased glut-1 and decreased vascular endothelial growth factor (VEGF) expression in affected skin. [1] The effect of cell-mediated cytotoxicity has been better defined at the biochemical level. [2] Systemic disease or involvement of other organ systems, unlike scleroderma, is not described, although many more authors are describing lichen sclerosus and scleroderma as closely related entities; many cases of coexistent lichen sclerosus/scleroderma having been reported.

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Epidemiology

Frequency

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

Race

Lichen sclerosus, both genital and extragenital, has no known racial predilection. A genetic predisposition, based on family clustering, is apparent. [3]

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 lichen sclerosus. Extragenital lichen sclerosus is rare in children.

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Prognosis

Prognosis is good for more acute genital cases, especially for those in pediatric age group that may resolve spontaneously. Prognosis for improvement is poor for extragenital cases and for chronic atrophic genital disease.

Lichen sclerosus 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 improve with puberty. In contrast, some authors suggest the rate of spontaneous resolution may be lower than 25%. [4] 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.

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Patient Education

Education relating to sexual dysfunction and dyspareunia may be required. Patients with genital lichen sclerosus should be educated on what changes (eg, ulceration) might indicate malignant transformation and mandate an immediate reevaluation.

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