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Lichen Sclerosus

Lichen sclerosus (LS) is a chronic, inflammatory skin disease of unknown cause, predominantly affecting the genital areas. It is not contagious and has a significant association with an increased risk of skin cancer. LS in adult women is generally incurable but manageable with treatment; untreated, it often worsens. In men, mild or intermediate disease affecting the foreskin or glans can often be cured through medical or surgical treatment.

Signs and Symptoms

Micrograph of lichen sclerosus showing the characteristic subepithelial sclerosus (right/bottom of image). H&E stain.
Micrograph of lichen sclerosus showing the characteristic subepithelial sclerosus (right/bottom of image). H&E stain.
Lichen sclerosus on an 82-year-old woman, showing an ivory white colouring in the vulva, and also stretching downward to the perineum.
Lichen sclerosus on an 82-year-old woman, showing an ivory white colouring in the vulva, and also stretching downward to the perineum.
Balanitis xerotica obliterans. 70 year old man.
Balanitis xerotica obliterans. 70 year old man.

LS may present without symptoms. Common symptoms include white patches, itching, pain, dyspareunia in genital LS, bruising, cracking, tearing, peeling, and hyperkeratosis. In women, the condition often affects the vulva and anus, while men may experience whitish patches, preputial stenosis, phimosis, and meatal stenosis. Non-genital skin manifestations include porcelain-white spots with follicular plugs and skin thinning.

Psychological Effect

Patients often experience distress, concerns with self-esteem, and sexual dysfunction. Counselling can help manage these feelings. The National Vulvodynia Association notes that vulvo-vaginal conditions can lead to isolation, hopelessness, low self-image, and depression.

Pathophysiology

The exact cause of LS is unknown, but several theories exist, including:

Genetic

A genetic component is suggested by high correlations of LS among twins and family members.

Autoimmunity

Autoimmune processes are implicated, with specific antibodies found in LS and a higher prevalence of other autoimmune diseases like type 1 diabetes, vitiligo, and thyroid disease.

Infection

Both bacterial and viral pathogens are considered potential causes. Borrelia burgdorferi, HPV, and hepatitis C are suspected. A link with Lyme disease is indicated by the presence of Borrelia burgdorferi in LS biopsy tissue.

Hormones

LS in females often occurs in prepubertal and postmenopausal women, suggesting hormonal influences, though evidence is limited.

Local Skin Changes

Sporadic findings suggest LS can be initiated by scarring or radiation.

Diagnosis

Micrograph of extragenital lichen sclerosus: epidermal atrophy, follicular plugging and basal vacuolisation, and sclerosis with initial homogenisation of collagen in the dermis.
Micrograph of extragenital lichen sclerosus: epidermal atrophy, follicular plugging and basal vacuolisation, and sclerosis with initial homogenisation of collagen in the dermis.

Diagnosis often involves a biopsy, revealing hyperkeratosis, atrophic epidermis, sclerosis of dermis, and lymphocyte activity. Clinical diagnosis of BXO is generally straightforward, but other dermatologic conditions like lichen planus and vitiligo can appear similar.

Treatment

Main Treatment

There is no definitive cure for LS. Behavioural changes, such as avoiding scratching and wearing loose clothing, are essential. Topical corticosteroids are the first-line treatment, proving effective in managing symptoms and potentially reducing cancer risk. Continuous use of corticosteroids is recommended to ensure symptom relief. Emollients are also advised to improve symptoms and can be used frequently. In males, circumcision may help but does not prevent flares or cancer.

Other Treatments

Carbon dioxide laser treatment improves symptoms but does not lower cancer risks. Platelet-rich plasma has shown significant improvement in quality of life in some studies.

Prognosis

LS can persist for a long time, with occasional spontaneous remission, particularly in young girls. There is an increased cancer risk associated with LS, particularly vulvar carcinoma in women and penile carcinoma in men.

Epidemiology

LS shows a bimodal age distribution in women, with peaks at 7.6 years and 60 years, and in boys, the average age is 9–11 years. In men, the most common age of incidence is 21–30 years.

History

LS was first described in 1887 by François Henri Hallopeau. The term lichen sclerosus et atrophicus was later shortened to lichen sclerosus in 1976 by the International Society for the Study of Vulvovaginal Disease (ISSVD).


Self-assessment MCQs (single best answer)

What is the main area affected by Lichen Sclerosus in adult women?



Which of the following is NOT a common symptom of Lichen Sclerosus?



What is the primary treatment for managing symptoms of Lichen Sclerosus?



Which pathogen has been linked to Lichen Sclerosus by the presence in biopsy tissue?



What is the increased risk associated with untreated Lichen Sclerosus?



Which autoimmune diseases are more prevalent in individuals with Lichen Sclerosus?



What is a common psychological effect experienced by patients with Lichen Sclerosus?



Which of the following is NOT a suggested theory for the cause of Lichen Sclerosus?



In men, which surgical procedure is often used to treat Lichen Sclerosus affecting the foreskin?



Who first described Lichen Sclerosus in 1887?



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Excellent content clearly explained.
SJ

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