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Lichen sclerosus in Adult
See also in: External and Internal Eye,Anogenital
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Lichen sclerosus in Adult

See also in: External and Internal Eye,Anogenital
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Contributors: Sabrina Nurmohamed MD, Stephanie Diamantis MD, Belinda Tan MD, PhD, Susan Burgin MD
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Synopsis

Lichen sclerosus (LS) is a chronic dermatosis characterized by an initial short inflammatory phase followed by chronic scarring and skin atrophy. It is a disease primarily of anogenital skin in both males and females. It is more common in females, with 2 peaks in age distribution: 1) prepubertal children, and 2) postmenopausal women. Childhood and adolescent cases in boys have increasingly been recognized.

The pathogenesis of LS is unknown, but an autoimmune mechanism with genetic predisposition is hypothesized. Some studies implicate Borrelia spp. and Epstein-Barr virus (EBV) infection, and trauma and hormonal influences have also been investigated.

Anogenital LS may be a chronic relapsing disease that may cause atrophy and scarring, functional impairment, and malignancy. Additionally, LS is one of the most common causes of chronic vulvar symptoms in adult females.

Clinically, over 85% of lesions are found on anogenital skin. In this location, LS usually presents as dry, tender, and severely pruritic atrophic white plaques. In females, characteristic sites include the clitoris and clitoral hood, interlabial sulci, labia minora, and perineum. Plaques can progress to cause fusion of labia minora to adjacent mucosa, and ultimately, fusion can involve clitoral hood and clitoris. Sclerosis of the vaginal introitus, with resultant dyspareunia, can also occur. Secondary erosion or ulceration may lead to burning and pain, especially after micturition. Difficulty in voiding may be seen in the later stages.

The majority of cases of male genital LS occur in uncircumcised men. The glans and prepuce are most frequently affected. Meatal involvement may occur. Scarring complications include urinary obstruction, ulceration, painful erection, and phimosis. There may also be diminished sensation of the glans and sexual dysfunction.

Squamous cell carcinoma can arise in untreated genital lesions.

LS may also occur on extragenital skin and rarely on the oral mucosa. The Koebner phenomenon, with development of LS at sites of trauma, has been reported. Extragenital LS is frequently asymptomatic; however, it can also be xerotic and pruritic. It most commonly presents as ivory-colored, atrophic papules and/or plaques with follicular accentuation.

For more information, see OMIM.

Codes

ICD10CM:
L90.0 – Lichen sclerosus et atrophicus

SNOMEDCT:
25674000 – Lichen Sclerosus et Atrophicus

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

There is often an overlap between extragenital LS and morphea (localized scleroderma); however, treatment remains the same. Lichen planus is an important differential for genital lesions.

Genital:
Extragenital:
Oral:

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 03/09/2017
Last Updated: 05/07/2019
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Lichen sclerosus in Adult
See also in: External and Internal Eye,Anogenital
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Lichen sclerosus : Eyelids, Glans of penis, Labia majora, Smooth plaque, White color, Inguinal region, Skin atrophy, Hardened skin
Clinical image of Lichen sclerosus
A shiny white plaque in the intergluteal fold.
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