Lichen Sclerosus

Question 1

What is lichen sclerosus?

Answer 1

Lichen sclerosus is a chronic, progressive skin disorder that most commonly affects the genitalia and perianal area.

Question 2

Lichen sclerosus is more common in who?

Answer 2

Lichen sclerosus is a chronic dermatological condition that is characterised by pruritus and irritation in the anogenital area. The condition is ten times more common in women

Question 3

Lichen sclerosus is thought to occur in 1 in … older woman.

Answer 3

Lichen sclerosus is thought to occur in 1 in 30 older woman.

Question 4

The cause of lichen sclerosus is …

Answer 4

The exact cause of lichen sclerosus is not completely understood, but it is thought to occur due to a combination of factors:

Genetic: lichen sclerosus may be seen in family members of patients with the condition
Immunological: lichen sclerosus is associated with other autoimmune conditions including alopecia areata, vitiligo, pernicious anaemia and diabetes mellitus. In addition, there is evidence of antibody formation against extracellular matrix protein 1 (ECM-1), which is a key dermal protein.
Hormonal: increased incidence during low oestrogen states
Infections: some infectious agents have been postulated to induce lichen sclerosis

Question 5

Lichen sclerosus predominantly affects the … region in 85-98% of patients.

Answer 5

Lichen sclerosus predominantly affects the anogenital region in 85-98% of patients.

Question 6

Symptoms of lichen sclerosus

Answer 6

Asymptomatic
Pruritus
Soreness or irritation
Dysuria
Dyspareunia (painful sexual intercourse)
Anal symptoms: bleeding, fissures, painful defecation, pruritus ani.
Painful erections (men)

Question 7

Signs of lichen sclerosus

Answer 7

A variety of lesions may occur in lichen sclerosis.

White atrophic plaques (typical change)
Haemorrhagic lesions (i.e. blood blister)
Bullae (fluid filled lesion >5mm)
Ulcers
Lichenification: thickening of skin due to excoriation (i.e. damage to skin surface due to scratching)
Adhesions and scarring: can lead to distortion of the labia minora, buried clitoris or introital stenosis (narrowing of vaginal entrance)
Phimosis: difficulty retracting foreskin in men
Meatal stenosis: narrowing of the external urethral meatus in men

Question 8

Extragenital lichen sclerosus

Answer 8

This may occur in up to 15% of cases (may be underestimate). Lesions tend to be asymptomatic.

Typical sites: thighs, breasts, wrists, shoulders, back, neck.
Appearance (white skin): typically white papules or atrophic papules
Appearance (dark skin): hypo- or hyperpigmentatory papules

Question 9

How is a formal diagnosis of lichen sclerosus made?

Answer 9

A formal diagnosis of lichen sclerosus is made from punch biopsy of the affected area.

Question 10

Punch biopsy for lichen sclerosus

Answer 10

A small punch biopsy from the vulva, or affected area, is usually sufficient to make the diagnosis. This may be completed in vulval or dermatology clinic.

Question 11

The classic appearance of lichen sclerosus is thinning of the epidermis with or without areas of …

Answer 11

The classic appearance of lichen sclerosus is thinning of the epidermis with or without areas of hyperkeratosis (abnormal thickening).

Question 12

There are a number of disorders that affect the vulva that may be confused with lichen sclerosus. Differentials?

Answer 12

Lichen planus: a similar pruritic vulval disorder that is characterised by well-demarcated, erythematous patches or erosions with a white lace-like pattern. Anal involvement is rare and this condition can involve the vagina.
Vulval dermatitis: refers to chronic irritation and pruritus of the vulva with associated erythema, fissuring and lichenification. Often involves the labia majora. Different types including contact dermatitis or atopic dermatitis.
Lichen simplex chronicus: this refers to squamous cell hyperplasia (increased cell growth) due to chronic vulval irritation. Typically develops due to vulval dermatitis and subsequent persistent rubbing and scratching.
Vulval psoriasis: usually appears as scaly erythematous plaques rather than white lesions.
Vulvovaginal candidiasis: refers to vaginal thrush that can cause an intensely itchy vulva with characteristic white discharge.

Question 13

Lichen sclerosus vs lichen planus

Answer 13

Lichen planus: a similar pruritic vulval disorder that is characterised by well-demarcated, erythematous patches or erosions with a white lace-like pattern. Anal involvement is rare and this condition can involve the vagina.

Lichen sclerosus predominantly affects the anogenital region in 85-98% of patients.
Lichen sclerosus is characterised by areas of thin, white skin over the anogenital region with or without distortion to the anatomy.

Question 14

Lichen sclerosus increases the risk …

Answer 14

Lichen sclerosus increases the risk of developing both vulval and penile squamous cell carcinoma.

Question 15

In women with lichen sclerosus , there is an increased risk of vulval squamous cell carcinoma, which is estimated to be …

Answer 15

In women, there is an increased risk of vulval squamous cell carcinoma, which is estimated to be <5%. There may be evidence of an early precursor lesion to squamous cell carcinoma known as vulvar intraepithelial neoplasia (VIN). VIN is also associated with human papilloma virus infection (HPV) and can be diagnosed on biopsy.

Question 16

Women with lichen sclerosus should be examined how often?

Answer 16

Women with lichen sclerosus should have the area of skin examined at least annually and non-resolving lesions biopsied, particularly hyperkeratotic areas.

Question 17

Conservative management of lichen sclerosus

Answer 17

Patient education and support is vital and patients should be referred to a clinician with a specialist interest. Basic management such as good hygiene (wash gently at least once a day), use of non-soap cleaners, avoidance of tight clothing and reduce scratching/rubbing is pertinent for all patients.

Question 18

Medical management of lichen sclerosus

Answer 18

The main treatment is topical steroids (e.g. clobetasol propionate 0.05% ointment) with emollients to manage dryness.

Question 19

Corticosteroids are highly effective for lichen sclerosus, therefore, treatment failure should warrant investigation for an alternative cause. This is particularly true before moving to second-line therapies.

Reasons for treatment failure:

Answer 19

Poor adherence or technique with topical steroids
Superinfection: commonly bacterial or fungal (e.g. candida)
Alternative diagnosis
Underlying malignancy
Atrophic vulvovaginitis due to menopause

Question 20

Patients with lichen sclerosus require long-term follow-up.

Answer 20

Due to the progressive nature of the disease, patients with lichen sclerosus are at risk of malignancy, chronic adhesions and scarring. They require long-term follow-up and ongoing treatment as needed.

Question 21

The diagnosis of lichen sclerosus is usually made clinically, based on the history and examination findings. Where there is doubt, what can confirm the diagnosis?

Answer 21

The diagnosis of lichen sclerosus is usually made clinically, based on the history and examination findings. Where there is doubt, a vulval biopsy can confirm the diagnosis.

Question 22

Changes affect the labia, perianal and perineal skin. There can be associated fissures, cracks, erosions or haemorrhages under the skin. The affected skin appears:

…

Answer 22

Changes affect the labia, perianal and perineal skin. There can be associated fissures, cracks, erosions or haemorrhages under the skin. The affected skin appears:

“Porcelain-white” in colour
Shiny
Tight
Thin
Slightly raised
There may be papules or plaques

Question 23

The critical complication of Lichen Sclerosus..

Answer 23

The critical complication to remember is a 5% risk of developing squamous cell carcinoma of the vulva.