Lichen sclerosus in Adult
See also in: External and Internal Eye,AnogenitalAlerts and Notices
Synopsis
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.
Codes
ICD10CM:L90.0 – Lichen sclerosus et atrophicus
SNOMEDCT:
895454001 – Lichen sclerosus
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:
- Lichen planus
- Lichen simplex chronicus
- Bowen disease / erythroplasia of Queyrat
- Sexual abuse should always be considered in individuals with scar-like lesions of the anogenital area.
- Scar
- Postmenopausal atrophy
- Extramammary Paget disease
- Zoon vulvitis / balanitis
- Bullous pemphigoid
- Recessive dystrophic epidermolysis bullosa (vulvar lesions)
- Junctional epidermolysis bullosa (vulvar lesions)
- Infiltrating basal cell carcinoma
- Chronic radiation dermatitis
- Anetoderma
- Atrophoderma of Pasini and Pierini
- Vitiligo
- Tinea versicolor
- Idiopathic guttate hypomelanosis
- Progressive macular hypomelanosis
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.Subscription Required
References
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Last Reviewed:03/08/2017
Last Updated:01/18/2022
Last Updated:01/18/2022
Lichen sclerosus in Adult
See also in: External and Internal Eye,Anogenital