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Extragenital lichen sclerosus

Heidi Jacobe, MD
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD


Lichen sclerosus (also known as lichen sclerosus et atrophicus) is a chronic inflammatory disorder characterized by the presence of porcelain white, atrophic plaques on the skin. Most cases of lichen sclerosus involve the female genitalia; extragenital manifestations occur in a minority of patients.

The epidemiology, clinical manifestations, and treatment of extragenital lichen sclerosus will be reviewed here. Anogenital lichen sclerosus is discussed separately. (See "Vulvar lichen sclerosus" and "Balanitis and balanoposthitis in adults", section on 'Lichen sclerosus or balanitis xerotica obliterans'.)


Extragenital manifestations are estimated to occur in approximately 15 to 20 percent of patients with lichen sclerosus [1]. Similar to genital lichen sclerosus, extragenital disease appears to be more common in women than in men. In a retrospective review, 17 out of 20 patients with histopathologic findings consistent with extragenital lichen sclerosus were female [2]. The disorder is rare in children.


The pathogenesis of lichen sclerosus is not well understood, and most information on this subject has been derived from studies in genital disease. Proposed etiologic factors for lichen sclerosus have included immune dysfunction, genetic predisposition, infectious agents, and trauma [3-12]. (See "Vulvar lichen sclerosus", section on 'Etiology'.)


Extragenital lichen sclerosus can occur in any location on the skin and infrequently appears in the oral cavity. Concomitant vulvar or penile lichen sclerosus may or may not be present. Conjunctival lesions do not occur.


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Literature review current through: Sep 2016. | This topic last updated: Sep 6, 2016.
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