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
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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
Patient Information for Lichen sclerosus in Adult
Overview
Lichen sclerosus refers to a type of inflammation, bleeding, thinning, scarring, and resultant white plaques of the mucous membrane and skin in the genital area, or possibly elsewhere on the body. Its origin is uncertain. There may be a genetic association among some family members.Lichen sclerosus lesions may resemble childhood or adult sexual abuse. Your doctor may need to rule out this possibility.
Who’s At Risk
Lichen sclerosus is reported 6 times more often in females than males. In females, it is most often genital and presents either before puberty or after menopause. Lichen sclerosus in males is almost always in uncircumcised boys and men.Women with genital lichen sclerosus may have a slightly greater risk of skin cancer.
Lichen sclerosus is rarely seen in infants.
Signs & Symptoms
Lichen sclerosus of the anogenital area begins with inflamed, dry, sore, and very itchy areas of genital skin. It may progress to painful, bleeding, red or purple lesions. Female symptoms can include pain during urination, defecation, or intercourse. In males, lesions may occur on the head and shaft of the penis. Other symptoms may include itching and painful urination, defecation, and erection. Impaired sensation of the penis may occur.Lichen sclerosus of other skin areas may occur without any symptoms, or may appear as dry, itchy skin patches. White, scar-like areas appear on the shoulders, arms, neck, and back. It may appear in tattoos or previously injured skin (surgical scars, trauma, burns).
Rarely does it occur in the mouth.
Self-Care Guidelines
Use only mild cleansers. Follow the directions of your health care provider to alleviate the pain, itch, and discomfort of the condition.When to Seek Medical Care
When the skin and mucous membranes begin to show signs of aggravated reddening, irritation, itching, and skin plaques, contact your doctor.If conditions worsen following your medical appointment, contact your doctor for follow-up. Lichen sclerosus, if left untreated, can cause irreversible damage to skin and mucous membranes.
Treatments
Your health care provider may perform tests to rule out other conditions or infections, or to confirm your diagnosis prior to starting treatment. Your health care provider may perform a biopsy to rule out malignancy and other white plaque conditions. A biopsy is not ordinarily performed on children, except in extreme cases.Treatment of symptoms, particularly itching, may be the first approach. Topical steroids have been used with great success. A number of other treatments have had mixed results, such as surgery, injections, cryotherapy, phototherapy, and other laser therapy. Circumcision is believed to be helpful for men.
Lichen sclerosus in Adult
See also in: External and Internal Eye,Anogenital